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Sample records for factor xiii deficiencies

  1. Recombinant factor XIII and congenital factor XIII deficiency: an update from human and animal studies

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    Inbal A

    2013-10-01

    Full Text Available Aida InbalThrombosis and Hemostasis Unit, Hematology Institute, Beilinson Hospital, Rabin Medical Center, Petach Tikva, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelAbstract: Factor XIII (FXIII is a protransglutaminase composed of two catalytic A subunits and two carrier B subunits. An intracellular form of FXIII is present in monocytes/macrophages and platelets as a homodimer of two A subunits. Following activation by thrombin, FXIII becomes plasma transglutaminase, which crosslinks γ-glutamyl-ε-lysine residues of fibrin chains and thereby stabilizes the fibrin clot. FXIII deficiency results in a moderate to severe hemorrhagic disorder, abnormal wound healing in about 30% of patients, and recurrent abortion in homozygous females. More than 800 cases of FXIII deficiency have been reported, most of them due to mutation in the FXIII-A gene, resulting in FXIII-A deficiency. Among mutations causing FXIII-A deficiency, 50% are missense mutations. Only 16 mutations in the FXIII-B gene have been published. Routine laboratory tests are normal in patients with FXIII deficiency, and the diagnosis is established by demonstration of decreased FXIII activity and antigen. Plasma-derived, virus-inactivated factor XIII concentrate is the treatment of choice. The low plasma levels of FXIII (about 5% required to control bleeding and its long half-life make monthly prophylactic therapy feasible. Recently, recombinant FXIII concentrate with a half-life similar to that of native FXIII has been developed and tested in a multinational clinical study. This new product appears to be safe and appropriate for lifelong prophylactic treatment of patients with FXIII-A deficiency.Keywords: recombinant FXIII concentrate, FXIII deficiency

  2. Medical and Surgical Management of Postpartum Hemorrhage in a Woman with Factor XIII Deficiency

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    Michael Cheng

    2016-01-01

    Full Text Available Factor XIII deficiency is a rare inherited coagulopathy. Factor XIII is the last clotting factor in the coagulation cascade to insure strength and stability to fibrin clots. Without this enzyme, the fibrous clot is unstable and nonresistant to fibrinolysis. Gravid women with this congenital disease are especially at risk for complications including miscarriages and hemorrhage without appropriate interventions. We present a case of a woman in her 20s with Factor XIII deficiency who was treated with cryoprecipitate and had a successful normal spontaneous vaginal delivery; subsequently, patient suffered from postpartum hemorrhage and consumptive coagulopathy due to consumption of Factor XIII, requiring emergency surgical intervention. Intraoperative management was challenged by an ethical dilemma involving the patient’s religious beliefs about not receiving blood. This paper will discuss the mechanism of Factor XIII and the medical and surgical management involved with this patient.

  3. New developments in the management of congenital Factor XIII deficiency [Corrigendum

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    Fadoo Z

    2013-07-01

    Full Text Available Fadoo Z, Merchant Q, Rehman KA. Journal of Blood Medicine. 2013;4:65–73.On page 67 the legend of Figure 1 incorrectly states "Reprinted from Song JW, Choi JR, Song KS, Rhee JH. Plasma factor XIII activity in patients with disseminated intravascular coagulation. Yonsei Med J. 2006;47(2:196–200." The correct statement is "Reprinted from Muszbek L, Bagoly Z, Cairo A, Peyvandi F. Novel aspects of factor XIII deficiency. Curr Opin Hematol. 2011;18(5:366–372."On page 69 the legend of Table 1 states "Reprinted from Song JW, Choi JR, Song KS, Rhee JH. Plasma factor XIII activity in patients with disseminated intravascular coagulation. Yonsei Med J. 2006;47(2:196–200." The correct statement is "Reprinted from Kohler HP, Ichinose A, Seitz R, Ariens RA, Muszbek L; Factor XIII and fibrinogen SSC subcommittee of the ISTH. Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost. 2011;9(7:1404–1406." Read the original article

  4. Molecular Basis of Inherited Factor XIII- A Deficiency among Patients from Sistan - Baluchestan

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    Hejazi Shabnam

    2010-03-01

    Full Text Available Background: Factor ХШ, the last zymogene in the clotting cascade, converts the loose fibrin polymer into a firm polymer. In the absence of factor ХШ the abnormal fibrin is soluble in acetic acid, as well as 5M urea. Factor ХШ is composed of 2 catalytic A subunit bounds and 2 B subunits as carriers (A2B2. The gene of A chain is located on chromosome 6. Factor ХШ deficiency is rare; with a prevalence of only 1 in 2 million in the general population. The overwhelming majority of cases are due to mutations in subunit A. The aim of this study was to detect the mutations of subunit A.Materials & Methods: In this study we investigated the molecular basis of inherited factor ХШ deficiency among 10 unrelated patients from Sistan and Balouchestan province in 2006. Mutations were detected by amplifying each exon. Those exons exhibiting the presence of heteroduplex by conformation sensitive gel electrophoresis (CSGE were selected for direct sequencing. Sequencing of mutations was carried out by restriction fragment length polymorphism (RFLP.Results: All patients had homologous subsitiation of TGG to CGG in exon 4 which led to change of arginine to tryptophan.Conclusion: The mutation found in this study was in the core domain of enzyme. It seems that the changs in electric charge and affinity of enzyme to substrate‚as a result decreases the level of factor XIII-A activity.

  5. Plasma factor XIII and platelet factor XIII in hyperlipaemia.

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    Cucuianu, M P; Miloszewski, K; Porutiu, D; Losowsky, M S

    1976-12-31

    Plasma factor XIII activity measured by a quantitative assay was found to be significantly higher in hypertriglyceridaemic patients (type IV and combined hyperlipoproteinaemia), as compared to normolipaemic controls. No such elevation in plasma factor XIII activity was found in patients with type Ha hyperlipaemia. Plasma pseudocholinesterase was found to parallel the elevated factor XIII activity in hypertriglyceridaemic subjects. In contrast, platelet factor XIII activity was not raised in hyperlipaemic subjects, and plasma factor XIII was found to be normal in a normolipaemic subjects with thrombocythaemia. It was concluded that there is no significant contribution from platelets to plasma factor XIII activity, and that the observed increase in plasma factor XIII in hypertriglyceridaemia results from enhanced hepatic synthesis of the enzyme.

  6. Original tandem duplication in FXIIIA gene with splicing site modification and four amino acids insertion causes factor XIII deficiency.

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    Louhichi, Nacim; Haj Salem, Ikhlass; Medhaffar, Moez; Miled, Nabil; Hadji, Ahmad F; Keskes, Leila; Fakhfakh, Faiza

    2017-04-01

    : Recessive mutations of F13A gene are reported to be responsible of FXIIIA subunit deficiency (FXIIIA). In all, some intronic nucleotide changes identified in this gene were investigated by in-silico analysis and occasionally supported by experimental data or reported in some cases as a polymorphism. To determine the molecular defects responsible of congenital factor XIII deficiency in Libyan patient, molecular analysis was performed by direct DNA sequencing of the coding regions and splice junctions of the FXIIIA subunit gene (F13A). A splicing minigene assay was used to study the effect of this mutation. Bioinformatics exploration was fulfilled to conceive consequences on protein. A 12-bp duplication straddling the border of intron 9 and exon 10 leads to two 3' acceptor splice sites, resulting in silencing of the downstream wild 3' splice site. It caused an in-frame insertion of 12 nucleotides into mRNA and four amino acids into protein. Bioinformatic analysis predicts that the insertion of four amino acids affects the site 3 of calcium binding site, which disturbs the smooth function of the FXIIIA peptide causing the factor XIII deficiency. This study showed that a small duplication seems to weaken the original 3' splice site and enhance the activation of a new splice site responsible for an alternative splicing. It would be interesting to examine the underlying molecular mechanism involved in this rearrangement.

  7. Detection of Factor XIII deficiency: data from multicentre exercises amongst UK NEQAS and PRO-RBDD project laboratories.

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    Jennings, I; Kitchen, S; Menegatti, M; Palla, R; Walker, I; Makris, M; Peyvandi, F

    2017-08-01

    FXIII deficiency is a rare bleeding disorders, and specific FXIII assays are recommended to detect this deficiency. We investigated the performance and accuracy of FXIII investigations in two exercises, comparing centres enrolled in the PRO-RBDD project (prospective data collection on patients with fibrinogen and Factor XIII deficiencies), and UK NEQAS BC centres. Samples from a FXIII deficient subject and a normal donor were sent to participating centres, to investigate for FXIII deficiency, and interpret their results. Median, coefficient of variation and range were determined. Results were returned from 98 UK NEQAS BC and 28 PRO-RBDD centres. Up to 40% of UK NEQAS BC and 52% of PRO-RBDD centres reported clot solubility results - with diagnostic errors by two NEQAS BC centres (false negatives for the FXIII deficient sample) and one PRO-RBDD centre (false positive for the normal sample). Over 70% of UK NEQAS BC centres and PRO-RBDD centres performed FXIII assays. Median results were similar between the two groups, with the exception of sample 3 in survey 2 (5.5 vs. 14.0 μ/dl for UK NEQAS BC and PRO-RBDD centres respectively, P UK NEQAS BC centres. Approximately 70% of centres now employ FXIII assays, complying with international recommendations. However, solubility tests continue to be used. Our data show this can be successful, depending on the sensitivity of the method in use. Diagnostic errors are made by centres using both solubility screens and FXIII assays, and laboratories should ensure good quality assurance procedures to improve diagnostic accuracy. © 2017 John Wiley & Sons Ltd.

  8. Central nervous system bleeding in pediatric patients with factor XIII deficiency: a study on 23 new cases.

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    Naderi, Majid; Alizadeh, Shaban; Kazemi, Ahmad; Tabibian, Shadi; Zaker, Farhad; Bamedi, Taregh; Kashani Khatib, Zahra; Dorgalaleh, Akbar

    2015-03-01

    Factor XIII (FXIII) deficiency is an extremely rare bleeding disorder, which has the highest incidence in Sistan and Baluchistan Province in Iran, compared to its overall incidence around the world. This disorder has different clinical manifestations ranging from mild bleeding tendency to lethal bleeding episodes including central nervous system (CNS) hemorrhage. The aim of this study was to evaluate the demographic data, pattern of CNS bleeding, and the role of plasminogen activator inhibitor-1 (PAI-1) (PAI-1) 4G/5G and thrombin activatable fibrinolysis inhibitor (TAFI) Thr325Ile polymorphisms in intracranial and extracranial hemorrhages in 23 new cases of FXIII-deficient subjects. This case-control study was conducted on 23 FXIII-deficient patients with CNS bleeding episodes and 23 patients as the control group with FXIII deficiency but without any history of CNS bleeding. Initially, to confirm the molecular defect, both groups were evaluated for the most frequently reported mutation of FXIII (Trp187Arg mutation) in a previous study in Sistan and Baluchistan Province. Then, demographic data, clinical manifestations, and pattern of CNS bleeding were determined. Eventually, the patients were assessed for PAI-14G/5G and TAFI Thr325Ile polymorphisms. The results of this study revealed that all the subjects (including the case and control groups) were homozygous for Trp187Arg mutation. Nineteen patients (82.6%) had intracranial hemorrhage (ICH) and four patients (17.4%) had extracranial hemorrhage (ECH). Intraparenchymal hemorrhage was the most common form of ICH (89.5%), and epidural hemorrhage was observed in two patients (10.5%). Anatomic regions in patients with intraparenchymal hemorrhage were temporal in six (35.3%), occipital in four (23.5%), diffused intraparenchymal in four (23.5%), temporal-occipital in two (11.8%), and subdural with temporal in one (5.9%) patient. We found that in the case group, 14 patients (60.8%) were homozygous for TAFI Thr325Ile

  9. First cases of severe congenital factor XIII deficiency in Southwestern Afghanistan in the vicinity of southeast of Iran.

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    Hosseini, Soudabeh; Dorgalaleh, Akbar; Bamedi, Taregh; Tavakol, Khanagha; Tabibian, Shadi; Naderi, Majid; Alizadeh, Shaban; Varmaghani, Bijan; Shamsizadeh, Morteza; Rahimizadeh, Aziz; Ebrahimi, Sharif

    2015-12-01

    Factor XIII deficiency (FXIIID) is an extremely rare bleeding disorder with the highest global incidence in southeast of Iran. Southwestern Afghanistan (Nimruz Province) is located near the border with Iran in the vicinity of Sistan and Baluchestan Province in southeast Iran, and there seems to be a high prevalence of FXIIID in Nimruz. Thus, this cross-sectional study was designed to assess the prevalence of FXIIID, molecular basis as well as clinical manifestations of FXIIID in Southwestern Afghanistan. During the course of the study, all patients suspected of FXIIID were clinically examined and assessed by routine coagulation tests, including bleeding time, activated partial thromboplastin time, prothrombin time, as well as platelet count and clot solubility test. Patients with normal routine coagulation tests, but abnormal clot solubility test, underwent further investigations by FXIII activity, as well as molecular analysis for FXIII-A gene mutation (Trp187Arg) by PCR-restriction fragment length polymorphism that confirmed by sequencing. Patients with confirmed FXIIID deficiency were registered to receive prophylaxis treatment. All data including demographic information, clinical manifestations, as well as therapeutic response and type and duration of treatment, were recorded, and the data were analyzed by SPSS software. In this cross-sectional study, we found five patients with abnormal clot solubility test, among whom two patients abandoned the study, whereas three patients remained for a more precise study. All the patients were residents of Zaranj city, the capital of Nimruz Province. All these patients had undetectable activity of FXIII, which indicates a severe deficiency. Molecular analysis of patients showed mutation of Trp187Arg in all of them. Hematoma was the most common clinical presentation leading to diagnosis of FXIIID in these patients (100%). Epistaxis (67%), gum bleeding (33%), and hematuria (33%) were other recurrent clinical presentations of

  10. Establishment of a prenatal diagnosis schedule as part of a prophylaxis program of factor XIII deficiency in the southeast of Iran.

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    Naderi, Majid; Reykande, Samira Esmaeili; Dorgalaleh, Akbar; Alizadeh, Shaban; Tabibian, Shadi; Einollahi, Nahid; Moghaddam, Ebrahim Miri

    2016-01-01

    Factor XIII deficiency (FXIIID) is an extremely rare bleeding disorder with a prevalence of 1 in 3 million in the general population. Compared to its global incidence, it has the greatest prevalence in Sistan and Baluchistan Province in the southeast of Iran. The high incidence of FXIIID in this region causes a high rate of morbidity and mortality among the affected individuals because of life-threatening episodes such as central nervous system (CNS) bleeding, umbilical cord bleeding, as well as miscarriage. CNS bleeding leads to a considerable number of neurological and behavioral complications. Therefore, we have designed an established prenatal diagnosis (PND) program to prevent the increasing incidence of life-threatening bleeding episodes and related complications among neonates with congenital FXIIID. This study was conducted from September 2013 to August 2014. A consent form was signed by the parents. Fetal sampling was done via abdominal chorionic villus sampling passage under local anesthesia and ultrasonic guidance within the first trimester of pregnancy. Fetal DNA was extracted, and PCR-restriction fragment length polymorphism was performed for the only reported mutation of FXIII (Trp187Arg) in the southeast of Iran. During the period of study, PND was performed on eight fetuses. Six fetuses were offspring of parental consanguineous marriages, and all of them had a positive family history of FXIIID. Seven out of the eight fetuses had a family member with CNS bleeding due to FXIIID. Four fetuses had a FXIIID-related death. One of the fetuses bore homozygous Trp187Arg mutation, whereas six were heterozygous, and one of the mothers gave birth to an unaffected fetus. To the best of our knowledge, PND is a possible solution to control high incidence of life-threatening episodes of FXIIID in southeast Iran.

  11. Tissue regenerating functions of coagulation factor XIII

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    Soendergaard, C; Kvist, P H; Seidelin, J B;

    2013-01-01

    The protransglutaminase factor XIII (FXIII) has recently gained interest within the field of tissue regeneration, as it has been found that FXIII significantly influences wound healing by exerting a multitude of functions. It supports haemostasis by enhancing platelet adhesion to damaged......-receptor 2 and the αVβ3 integrin is important for angiogenesis supporting formation of granulation tissue. Chronic inflammatory conditions involving bleeding and activation of the coagulation cascade have been shown to lead to reduced FXIII levels in plasma. Of particular importance for this review...

  12. [Implant fixation strength and osseointegration following systemic administration of recombinant factor XIII and factor XIII concentrate. Animal experiment with implant fixation strength and osseointegration of porous surface implants].

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    Kienapfel, H; Wilke, A; Dörner, P; Jürgensen, R; Prinz, H; Hettel, A; Swain, R; Griss, P

    1995-01-01

    30 cylindrical commercially pure titanium fiber porous coated Ti6A14V implants were inserted press-fit into the proximal humeral portion of 30 sheep humeri to determine the systemic effect of recombinant factor XIII and placenta-derived factor XIII concentrate on bone ingrowth and on strength of fixation. For both the recombinant factor XIII and the factor XIII concentrate group the volume fraction of bone ingrowth and the strength of fixation was higher when compared with the control specimens. However the difference was only significant for the factor XIII concentrate group.

  13. 21 CFR 866.5330 - Factor XIII, A, S, immuno-logical test system.

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    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Factor XIII, A, S, immuno-logical test system. 866.5330 Section 866.5330 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN....5330 Factor XIII, A, S, immuno-logical test system. (a) Identification. A factor XIII, A,...

  14. Recombinant and nonrecombinant factor XIII and its effect on bone ingrowth and strength of fixation.

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    Kienapfel, H; Swain, R; Hettel, A; Wilke, A; Koller, M; Griss, P

    1997-01-01

    Thirty cylindrical, commercially pure, titanium fiber, porous-coated Ti6Al4V implants were inserted press-fit into the proximal humeral portion of 30 sheep humeri to determine the systemic effect of recombinant factor XIII and placenta-derived factor XIII concentrate on bone ingrowth and strength of fixation. For both the recombinant factor XIII and the factor XIII concentrate group, the volume of bone ingrowth and the strength of fixation were higher than for the control specimens. However, the difference was only significant for the factor XIII concentrate group.

  15. Genetics Home Reference: factor XIII deficiency

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    ... percent of normal), and they may experience abnormal bleeding after surgery, dental work, or major trauma. However, most people who carry one mutated copy of the F13A1 or F13B gene do not have abnormal bleeding episodes under normal circumstances, and so they never ...

  16. Factor XIII Deficiency and Intracranial Hemorrhage

    OpenAIRE

    1990-01-01

    A 38 month old boy with excessive bleeding following circumcision as a newborn and two episodes of intracranial hemorrhage at four months and at 85 months of age is reported from the Scott and White Clinic, Temple, TX.

  17. Factor XIII Va134Leu and the risk of venous thromboembolism in factor V Leiden carriers

    NARCIS (Netherlands)

    Franco, RF; Middeldorp, S; Meinardi, [No Value; van Pampus, ECM; Reitsma, PH

    2000-01-01

    A mutation in factor XIII (Val34Leu) was reported to protect against Venous thromboembolism. We evaluated the effect of Val34Leu on thrombotic risk in 352 factor V Leiden carriers who were first-degree relatives of 132 thrombotic propositi carrying factor V Leiden. The total observation period was

  18. Factor XIII Va134Leu and the risk of venous thromboembolism in factor V Leiden carriers

    NARCIS (Netherlands)

    Franco, RF; Middeldorp, S; Meinardi, [No Value; van Pampus, ECM; Reitsma, PH

    2000-01-01

    A mutation in factor XIII (Val34Leu) was reported to protect against Venous thromboembolism. We evaluated the effect of Val34Leu on thrombotic risk in 352 factor V Leiden carriers who were first-degree relatives of 132 thrombotic propositi carrying factor V Leiden. The total observation period was 2

  19. Ulcerative colitis and Crohn's disease: factor XIII, inflammation and haemostasis.

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    Seitz, R; Leugner, F; Katschinski, M; Immel, A; Kraus, M; Egbring, R; Göke, B

    1994-01-01

    An important role has been ascribed to plasma factor XIII (FXIII) in inflammation and wound healing. FXIII is necessary for fibrin stabilization and interacts with connective tissue and adhesive proteins and cells. In a prospective study, FXIII activity and parameters of coagulation, fibrinolysis and inflammation, were determined in patients with ulcerative colitis (UC; 13 active, 22 moderate) and Crohn's disease (CD; 36 active, 45 moderate). FXIII levels were lower in active than in moderate UC and CD, and were < 70% of normal values in 7/13 patients with active UC, and in 7/36 patients with active CD, although the median values did not fall below the normal range. FXIII was somewhat higher in active UC patients responding to therapy. The FXIII levels were widely scattered, and low values appear to be due to greatly enhanced turnover. A correlation between FXIII and the systemic levels of markers of activation of haemostasis and inflammation was lacking, but there was a correlation with the extent of bowel involvement. FXIII levels were lower in the patients with involvement beyond the sigmoid colon in CU (p = 0.0045), and both small and large bowel segments in CD (p = 0.0223) patients. This points to local consumption and/or loss of FXIII within the inflamed tissue, and provides an argument for FXIII substitution in the treatment of acute episodes of inflammatory bowel diseases.

  20. Fibrinolytic and factor XIII activity in subarachnoid hemorrhage Actividad fíbrinolítica y del factor XIII en la hemorragia subaracnoidea

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    Luis A. Pliego

    1997-09-01

    Full Text Available The balance between fibrinolytic activity and coagulation mechanisms seems to play an important role in the rebleeding of a subarachnoid hemorrhage (SAH due to aneurysmatic rupture. In the present paper we describe our findings in a group of patients (n10 with S AH. The plasmatic levels of fibrinogen and their degradation products (FDP, APTT, prothrombin activity and factor XIII were determined within 72 hours of initial bleeding, or of eventual rebleeding. Factor XIII activity in the first bleeding was 82.1 ±4%, while the levels of FDP were 3.8±lmg/ml. In patients presenting rebleeding (n4, Factor XIII activity was 67.3±4.5% the day it manifested, which is significantly less than the values previously observed (pEl balance entre la actividad fíbrinolítica y los mecanismos de la coagulación juegan un rol importante en el resangrado de una hemorragia subaracnoidea debido a ruptura aneurismástica. En el presente estúdio se describen nuestros hallazgos en un grupo de pacientes (n10 con hemorragia subaracnoidea (SAH. Los niveles plasmaticos de fibrinogeno y sus productos de degradation (FDP, la actividad de protrombina (APTT y de factor XIII fueron determinados dentro de las 72 hs, dei sangrado inicial y ante el eventual resangrado. La actividad de factor XIII en el primer sangrado fue de 82,1±4%, mientras el nivel de FDP fue de 3,8±lmg/ml. En aquellos pacientes que resangraron (n4, la actividad de factor XIII fue de 67,3+4,5%, el dia del resangrado, el cual es significativamente menor que los valores previamente observados (p<0.01, mientras que los niveles de FDP fueron 4,1± 2mg/ml. La reduction de la actividad de factor XIII sugiere un importante rol de este en la establilidad del coágulo en el sitio de ruptura aneurismática, siendo posible atribuir un valor predicitivo a la reduction de su actividad.

  1. Factor XIII activity mediates red blood cell retention in venous thrombi

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    Aleman, Maria M.; Byrnes, James R.; Wang, Jian-Guo; Tran, Reginald; Lam, Wilbur A.; Di Paola, Jorge; Mackman, Nigel; Degen, Jay L.; Flick, Matthew J.; Wolberg, Alisa S.

    2014-01-01

    Venous thrombi, fibrin- and rbc-rich clots triggered by inflammation and blood stasis, underlie devastating, and sometimes fatal, occlusive events. During intravascular fibrin deposition, rbc are thought to become passively trapped in thrombi and therefore have not been considered a modifiable thrombus component. In the present study, we determined that activity of the transglutaminase factor XIII (FXIII) is critical for rbc retention within clots and directly affects thrombus size. Compared with WT mice, mice carrying a homozygous mutation in the fibrinogen γ chain (Fibγ390–396A) had a striking 50% reduction in thrombus weight due to reduced rbc content. Fibrinogen from mice harboring the Fibγ390–396A mutation exhibited reduced binding to FXIII, and plasma from these mice exhibited delayed FXIII activation and fibrin crosslinking, indicating these residues mediate FXIII binding and activation. FXIII-deficient mice phenocopied mice carrying Fibγ390–396A and produced smaller thrombi with fewer rbc than WT mice. Importantly, FXIII-deficient human clots also exhibited reduced rbc retention. The addition of FXIII to FXIII-deficient clots increased rbc retention, while inhibition of FXIII activity in normal blood reduced rbc retention and produced smaller clots. These findings establish the FXIII-fibrinogen axis as a central determinant in venous thrombogenesis and identify FXIII as a potential therapeutic target for limiting venous thrombosis. PMID:24983320

  2. Knee hemarthrosis after arthroscopic surgery in an athlete with low factor XIII activity

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    Tsujii Akira

    2012-10-01

    Full Text Available Abstract We report a thirteen-year-old tennis player with knee hemarthrosis caused by low factor XIII activity. She visited our hospital because of medial peripatellar pain for two years. Although there was no abnormal sign in X-ray or MRI, diagnostic arthroscopy was performed. It revealed some cartilage debris, medial plica and complete septum of suprapatellar plica. Removing the debris by washing out and resecting the medial plica, she could return to play tennis without perioperative symptom. Two months after the first operation, her knee got swelling without any apparent cause. Since 20 ml blood was aspirated twice and MRI revealed suprapatellar mass, we performed arthroscopy again. Suprapatellar mass was old blood clot covered with complete suprapatellar plica. Resection of suprapatellar plica and washing out blood clot were performed, and severe postoperative hemarthrosis was progressively occurred. As factor XIII level was 54% preoperatively, we diagnosed that this condition was caused by low activity level of the factor and administered factor XIII concentrates. The level got improved to 129% and then hemarthrosis gradually relieved. She had no signs of recurrence. We should keep in mind of low factor XIII activity case in case of unexplained postoperative hemarthrosis after arthroscopy because consumption of the factor might promote this condition.

  3. Knee hemarthrosis after arthroscopic surgery in an athlete with low factor XIII activity.

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    Tsujii, Akira; Tanaka, Yoshinari; Yonetani, Yasukazu; Shiozaki, Yoshiki; Tomiyama, Yoshiaki; Horibe, Shuji

    2012-10-02

    We report a thirteen-year-old tennis player with knee hemarthrosis caused by low factor XIII activity. She visited our hospital because of medial peripatellar pain for two years. Although there was no abnormal sign in X-ray or MRI, diagnostic arthroscopy was performed. It revealed some cartilage debris, medial plica and complete septum of suprapatellar plica. Removing the debris by washing out and resecting the medial plica, she could return to play tennis without perioperative symptom. Two months after the first operation, her knee got swelling without any apparent cause. Since 20 ml blood was aspirated twice and MRI revealed suprapatellar mass, we performed arthroscopy again. Suprapatellar mass was old blood clot covered with complete suprapatellar plica. Resection of suprapatellar plica and washing out blood clot were performed, and severe postoperative hemarthrosis was progressively occurred. As factor XIII level was 54% preoperatively, we diagnosed that this condition was caused by low activity level of the factor and administered factor XIII concentrates. The level got improved to 129% and then hemarthrosis gradually relieved. She had no signs of recurrence. We should keep in mind of low factor XIII activity case in case of unexplained postoperative hemarthrosis after arthroscopy because consumption of the factor might promote this condition.

  4. Coated platelets function in platelet-dependent fibrin formation via integrin αIIbβ3 and transglutaminase factor XIII.

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    Mattheij, Nadine J A; Swieringa, Frauke; Mastenbroek, Tom G; Berny-Lang, Michelle A; May, Frauke; Baaten, Constance C F M J; van der Meijden, Paola E J; Henskens, Yvonne M C; Beckers, Erik A M; Suylen, Dennis P L; Nolte, Marc W; Hackeng, Tilman M; McCarty, Owen J T; Heemskerk, Johan W M; Cosemans, Judith M E M

    2016-04-01

    Coated platelets, formed by collagen and thrombin activation, have been characterized in different ways: i) by the formation of a protein coat of α-granular proteins; ii) by exposure of procoagulant phosphatidylserine; or iii) by high fibrinogen binding. Yet, their functional role has remained unclear. Here we used a novel transglutaminase probe, Rhod-A14, to identify a subpopulation of platelets with a cross-linked protein coat, and compared this with other platelet subpopulations using a panel of functional assays. Platelet stimulation with convulxin/thrombin resulted in initial integrin α(IIb)β3 activation, the appearance of a platelet population with high fibrinogen binding, (independently of active integrins, but dependent on the presence of thrombin) followed by phosphatidylserine exposure and binding of coagulation factors Va and Xa. A subpopulation of phosphatidylserine-exposing platelets bound Rhod-A14 both in suspension and in thrombi generated on a collagen surface. In suspension, high fibrinogen and Rhod-A14 binding were antagonized by combined inhibition of transglutaminase activity and integrin α(IIb)β3 Markedly, in thrombi from mice deficient in transglutaminase factor XIII, platelet-driven fibrin formation and Rhod-A14 binding were abolished by blockage of integrin α(IIb)β3. Vice versa, star-like fibrin formation from platelets of a patient with deficiency in α(IIb)β3(Glanzmann thrombasthenia) was abolished upon blockage of transglutaminase activity. We conclude that coated platelets, with initial α(IIb)β3 activation and high fibrinogen binding, form a subpopulation of phosphatidylserine-exposing platelets, and function in platelet-dependent star-like fibrin fiber formation via transglutaminase factor XIII and integrin α(IIb)β3.

  5. Knee hemarthrosis after arthroscopic surgery in an athlete with low factor XIII activity

    OpenAIRE

    Tsujii Akira; Tanaka Yoshinari; Yonetani Yasukazu; Shiozaki Yoshiki; Tomiyama Yoshiaki; Horibe Shuji

    2012-01-01

    Abstract We report a thirteen-year-old tennis player with knee hemarthrosis caused by low factor XIII activity. She visited our hospital because of medial peripatellar pain for two years. Although there was no abnormal sign in X-ray or MRI, diagnostic arthroscopy was performed. It revealed some cartilage debris, medial plica and complete septum of suprapatellar plica. Removing the debris by washing out and resecting the medial plica, she could return to play tennis without perioperative sympt...

  6. Factor XIII Transglutaminase Supports the Resolution of Mucosal Damage in Experimental Colitis.

    Directory of Open Access Journals (Sweden)

    Christina Andersson

    Full Text Available The thrombin-activated transglutaminase factor XIII (FXIII that covalently crosslinks and stablizes provisional fibrin matrices is also thought to support endothelial and epithelial barrier function and to control inflammatory processes. Here, gene-targeted mice lacking the FXIII catalytic A subunit were employed to directly test the hypothesis that FXIII limits colonic pathologies associated with experimental colitis. Wildtype (WT and FXIII-/- mice were found to be comparable in their initial development of mucosal damage following exposure to dextran sulfate sodium (DSS challenge. However, unlike FXIII-sufficient mice, FXIII-deficient cohorts failed to efficiently resolve colonic inflammatory pathologies and mucosal damage following withdrawal of DSS. Consistent with prior evidence of ongoing coagulation factor activation and consumption in individuals with active colitis, plasma FXIII levels were markedly decreased in colitis-challenged WT mice. Treatment of colitis-challenged mice with recombinant human FXIII-A zymogen significantly mitigated weight loss, intestinal bleeding, and diarrhea, regardless of whether cohorts were FXIII-sufficient or were genetically devoid of FXIII. Similarly, both qualitative and quantitative microscopic analyses of colonic tissues revealed that exogenous FXIII improved the resolution of multiple colitis disease parameters in both FXIII-/- and WT mice. The most striking differences were seen in the resolution of mucosal ulceration, the most severe histopathological manifestation of DSS-induced colitis. These findings directly demonstrate that FXIII is a significant determinant of mucosal healing and clinical outcome following inflammatory colitis induced mucosal injury and provide a proof-of-principle that clinical interventions supporting FXIII activity may be a means to limit colitis pathology and improve resolution of mucosal damage.

  7. Factor XIII Transglutaminase Supports the Resolution of Mucosal Damage in Experimental Colitis

    Science.gov (United States)

    Andersson, Christina; Kvist, Peter H.; McElhinney, Kathryn; Baylis, Richard; Gram, Luise K.; Pelzer, Hermann; Lauritzen, Brian; Holm, Thomas L.; Hogan, Simon; Wu, David; Turpin, Brian; Miller, Whitney; Palumbo, Joseph S.

    2015-01-01

    The thrombin-activated transglutaminase factor XIII (FXIII) that covalently crosslinks and stablizes provisional fibrin matrices is also thought to support endothelial and epithelial barrier function and to control inflammatory processes. Here, gene-targeted mice lacking the FXIII catalytic A subunit were employed to directly test the hypothesis that FXIII limits colonic pathologies associated with experimental colitis. Wildtype (WT) and FXIII-/- mice were found to be comparable in their initial development of mucosal damage following exposure to dextran sulfate sodium (DSS) challenge. However, unlike FXIII-sufficient mice, FXIII-deficient cohorts failed to efficiently resolve colonic inflammatory pathologies and mucosal damage following withdrawal of DSS. Consistent with prior evidence of ongoing coagulation factor activation and consumption in individuals with active colitis, plasma FXIII levels were markedly decreased in colitis-challenged WT mice. Treatment of colitis-challenged mice with recombinant human FXIII-A zymogen significantly mitigated weight loss, intestinal bleeding, and diarrhea, regardless of whether cohorts were FXIII-sufficient or were genetically devoid of FXIII. Similarly, both qualitative and quantitative microscopic analyses of colonic tissues revealed that exogenous FXIII improved the resolution of multiple colitis disease parameters in both FXIII-/- and WT mice. The most striking differences were seen in the resolution of mucosal ulceration, the most severe histopathological manifestation of DSS-induced colitis. These findings directly demonstrate that FXIII is a significant determinant of mucosal healing and clinical outcome following inflammatory colitis induced mucosal injury and provide a proof-of-principle that clinical interventions supporting FXIII activity may be a means to limit colitis pathology and improve resolution of mucosal damage. PMID:26098308

  8. Characterization of the gene for the a subunit of human factor XIII (plasma transglutaminase), a blood coagulation factor

    Energy Technology Data Exchange (ETDEWEB)

    Ichinose, A.; Davie, E.W. (Univ. of Washington, Seattle (USA))

    1988-08-01

    Factor XIII (plasma transglutaminase, fibrin stabilizing factor) is a glycoprotein that circulates in blood as a tetramer (a{sub 2}b{sub 2}) consisting of two a and two b subunits. The primary structures of the a and b subunits of human factor XIII have been reported by a combination of cDNA cloning and amino acid sequence analysis. To establish the gene structure of the a subunit for factor XIII, several human genomic libraries were screened by using the cDNA encoding the a subunit as a probe. Among {approx}5 {times} 10{sup 7} recombinant phage, 121 have been shown to contain an insert encoding a portion of the a subunit. Twenty-five unique clones were than characterized by restriction mapping, Southern blotting, and DNA sequencing. Overlapping clones encoding the a subunit of factor XIII span >160 kilobases. DNA sequence analysis revealed that the activation peptide released by thrombin, the active site cysteine region, the two putative calcium-binding regions, and the thrombin cleavage site leading to inactivation are encoded by separate exons. This suggest that the introns may separate the a subunit into functional and structural domains. A comparison of the amino acid sequence deduced from the genomic DNA sequence with those deduced from cDNA or determined by amino acid sequence analysis of the plasma and placental proteins revealed apparent amino acid polymorphisms in six positions of the polypeptide chain of the a subunit.

  9. Factor II deficiency

    Science.gov (United States)

    ... if one or more of these factors are missing or are not functioning like they should. Factor II is one such coagulation factor. Factor II deficiency runs in families (inherited) and is very rare. Both parents must ...

  10. Factor VII deficiency

    Science.gov (United States)

    ... if one or more of these factors are missing or are not functioning like they should. Factor VII is one such coagulation factor. Factor VII deficiency runs in families (inherited) and is very rare. Both parents must ...

  11. Genetic Factors Influencing Coagulation Factor XIII B-Subunit Contribute to Risk of Ischemic Stroke.

    Science.gov (United States)

    Hanscombe, Ken B; Traylor, Matthew; Hysi, Pirro G; Bevan, Stephen; Dichgans, Martin; Rothwell, Peter M; Worrall, Bradford B; Seshadri, Sudha; Sudlow, Cathie; Williams, Frances M K; Markus, Hugh S; Lewis, Cathryn M

    2015-08-01

    Abnormal coagulation has been implicated in the pathogenesis of ischemic stroke, but how this association is mediated and whether it differs between ischemic stroke subtypes is unknown. We determined the shared genetic risk between 14 coagulation factors and ischemic stroke and its subtypes. Using genome-wide association study results for 14 coagulation factors from the population-based TwinsUK sample (N≈2000 for each factor), meta-analysis results from the METASTROKE consortium ischemic stroke genome-wide association study (12 389 cases, 62 004 controls), and genotype data for 9520 individuals from the WTCCC2 ischemic stroke study (3548 cases, 5972 controls-the largest METASTROKE subsample), we explored shared genetic risk for coagulation and stroke. We performed three analyses: (1) a test for excess concordance (or discordance) in single nucleotide polymorphism effect direction across coagulation and stroke, (2) an estimation of the joint effect of multiple coagulation-associated single nucleotide polymorphisms in stroke, and (3) an evaluation of common genetic risk between coagulation and stroke. One coagulation factor, factor XIII subunit B (FXIIIB), showed consistent effects in the concordance analysis, the estimation of polygenic risk, and the validation with genotype data, with associations specific to the cardioembolic stroke subtype. Effect directions for FXIIIB-associated single nucleotide polymorphisms were significantly discordant with cardioembolic disease (smallest P=5.7×10(-04)); the joint effect of FXIIIB-associated single nucleotide polymorphisms was significantly predictive of ischemic stroke (smallest P=1.8×10(-04)) and the cardioembolic subtype (smallest P=1.7×10(-04)). We found substantial negative genetic covariation between FXIIIB and ischemic stroke (rG=-0.71, P=0.01) and the cardioembolic subtype (rG=-0.80, P=0.03). Genetic markers associated with low FXIIIB levels increase risk of ischemic stroke cardioembolic subtype. © 2015 The

  12. Factor V deficiency

    Science.gov (United States)

    ... When certain blood clotting factors are low or missing, your blood does not clot properly. Factor V deficiency is rare. It may be caused by: A defective Factor V gene passed down through families (inherited) An antibody that interferes with normal Factor ...

  13. [A case of pancreatic and duodenal fistula after total gastrectomy successfully treated with coagulation factor XIII].

    Science.gov (United States)

    Nishino, Hitoe; Kojima, Kazuhiro; Oshima, Hirokazu; Nakagawa, Koji; Fumura, Masao; Kikuchi, Norio

    2013-11-01

    Pancreatic fistula( PF) is a challenging postoperative complication. We report a case of PF following gastrectomy successfully treated using intravenous coagulation factor XIII( FXIII).A 78-year-old man with early gastric cancer underwent total gastrectomy with Roux-en-Y reconstruction. PF developed postoperatively, following which, leakage from the duodenal stump was observed. Percutaneous drainage and re-operative surgery were performed. A somatostatin analogue, antibiotic drugs, and gabexate mesilate were administrated along with nutritional support. The pancreatic and duodenal fistula had been producing duodenal juice for over 30 days since the re-operative surgery. As suspected, reduced FXIII activity was confirmed in the patient. After administering FXIII for 5 days, the amount of duodenal juice from the fistula markedly reduced, and the fistula closed immediately afterwards. The results of our study suggest that administration of FXIII could be a reasonable and effective treatment for patients with pancreatic or/and enterocutaneous fistula who are resistant to standard treatments.

  14. Platelet factor XIII increases the fibrinolytic resistance of platelet-rich clots by accelerating the crosslinking of alpha 2-antiplasmin to fibrin

    Science.gov (United States)

    Reed, G. L.; Matsueda, G. R.; Haber, E.

    1992-01-01

    Platelet clots resist fibrinolysis by plasminogen activators. We hypothesized that platelet factor XIII may enhance the fibrinolytic resistance of platelet-rich clots by catalyzing the crosslinking of alpha 2-antiplasmin (alpha 2AP) to fibrin. Analysis of plasma clot structure by polyacrylamide gel electrophoresis and immunoblotting revealed accelerated alpha 2AP-fibrin crosslinking in platelet-rich compared with platelet-depleted plasma clots. A similar study of clots formed with purified fibrinogen (depleted of factor XIII activity), isolated platelets, and specific factor XIII inhibitors indicated that this accelerated crosslinking was due to the catalytic activity of platelet factor XIII. Moreover, when washed platelets were aggregated by thrombin, there was evidence of platelet factor XIII-mediated crosslinking between platelet alpha 2AP and platelet fibrin(ogen). Specific inhibition (by a monoclonal antibody) of the alpha 2AP associated with washed platelet aggregates accelerated the fibrinolysis of the platelet aggregate. Thus in platelet-rich plasma clots, and in thrombin-induced platelet aggregates, platelet factor XIII actively formed alpha 2AP-fibrin crosslinks, which appeared to enhance the resistance of platelet-rich clots to fibrinolysis.

  15. Factor XIII B Subunit Polymorphisms and the Risk of Coronary Artery Disease

    Directory of Open Access Journals (Sweden)

    Zoltán A. Mezei

    2015-01-01

    Full Text Available The aim of the case-control study was to explore the effect of coagulation factor XIII (FXIII B subunit (FXIII-B polymorphisms on the risk of coronary artery disease, and on FXIII levels. In the study, 687 patients admitted for coronary angiography to investigate suspected coronary artery disease and 994 individuals representing the Hungarian population were enrolled. The patients were classified according to the presence of significant coronary atherosclerosis (CAS and history of myocardial infarction (MI. The F13B gene was genotyped for p.His95Arg and for intron K nt29756 C>G polymorphisms; the latter results in the replacement of 10 C-terminal amino acids by 25 novel amino acids. The p.His95Arg polymorphism did not influence the risk of CAS or MI. The FXIII-B intron K nt29756 G allele provided significant protection against CAS and MI in patients with a fibrinogen level in the upper tertile. However, this effect prevailed only in the presence of the FXIII-A Leu34 allele, and a synergism between the two polymorphisms was revealed. Carriers of the intron K nt29756 G allele had significantly lower FXIII levels, and FXIII levels in the lower tertile provided significant protection against MI. It is suggested that the protective effect of the combined polymorphisms is related to decreased FXIII levels.

  16. Impaired Activity of Blood Coagulant Factor XIII in Patients with Necrotizing Enterocolitis.

    Science.gov (United States)

    Tao, Guo-Zhong; Liu, Bo; Zhang, Rong; Liu, Gigi; Abdullah, Fizan; Harris, Mary Cay; Brandt, Mary L; Ehrenkranz, Richard A; Bowers, Corinna; Martin, Camilia R; Moss, R Lawrence; Sylvester, Karl G

    2015-08-17

    Necrotizing enterocolitis (NEC) is the most common gastrointestinal (GI) medical/surgical emergency of the newborn and a leading cause of preterm neonate morbidity and mortality. NEC is a challenge to diagnose since it often shares similar clinical features with neonatal sepsis. In the present study, plasma protein profiling was compared among NEC, sepsis and control cohorts using gel electrophoresis, immunoblot and mass spectrometry. We observed significant impairment in the formation of fibrinogen-γ dimers (FGG-dimer) in the plasma of newborns with NEC that could efficiently differentiate NEC and sepsis with a high level of sensitivity and specificity. Interestingly, the impaired FGG-dimer formation could be restored in NEC plasma by the addition of exogenous active factor XIII (FXIII). Enzymatic activity of FXIII was determined to be significantly lower in NEC subject plasma for crosslinking FGG when compared to sepsis. These findings demonstrate a potential novel biomarker and related biologic mechanism for diagnosing NEC, as well as suggest a possible therapeutic strategy.

  17. Novel aspects of blood coagulation factor XIII. I. Structure, distribution, activation, and function

    Energy Technology Data Exchange (ETDEWEB)

    Muszbek, L.; Adany, R. [Univ. Medical School of Debrecen (Hungary); Mikkola, H. [Univ. of Helsinki (Finland)

    1996-10-01

    Blood coagulation factor XIII (FXIII) is a protransglutaminase that becomes activated by the concerted action of thrombin and Ca{sup 2+} in the final stage of the clotting cascade. In addition to plasma, FXIII also occurs in platelets, monocytes, and monocyte-derived macrophages. While the plasma factor is a heterotetramer consisting of paired A and B subunits (A{sub 2}B{sub 2}), its cellular counterpart lacks the B subunits and is a homodimer of potentially active A subunits (A{sub 2}). The gene coding for the A and B subunits has been localized to chromosomes 6p24-25 and 1q31-32.1, respectively. The genomic as well as the primary protein structure of both subunits has been established. Plasma FXIII circulates in association with its substrate precursor, fibrinogen. Fibrin(ogen) has an important regulatory role in the activation of plasma FXIII, for instance the proteolytic removal of activation peptide by thrombin, the dissociation of subunits A and B, and the exposure of the originally buried active site on the free A subunits. The end result of this process is the formation of an active transglutaminase, which crosslinks peptide chains through {epsilon}({gamma}-glutamyl)lysyl isopeptide bonds. The protein substrates of activated FXIII include components of the clotting-fibrinolytic system, adhesive and contractile proteins. The main physiological function of plasma FXIII is to cross-link fibrin and protect it from the fibrinolytic enzyme plasmin. The latter effect is achieved mainly by covalently linking {alpha}{sub 2} antiplasmin, the most potent physiological inhibitor of plasmin, to fibrin. Plasma FXIII seems to be involved in wound healing and tissue repair, and it is essential to maintaining pregnancy. Cellular FXIII, if exposed to the surface of the cells, might support or perhaps take over the hemostatic functions of plasma FXIII; however, its intracellular role has remained mostly unexplored. 328 refs., 4 figs.

  18. Local activation of coagulation factor XIII reduces systemic complications and improves the survival of mice after Streptococcus pyogenes M1 skin infection.

    Science.gov (United States)

    Deicke, Christin; Chakrakodi, Bhavya; Pils, Marina C; Dickneite, Gerhard; Johansson, Linda; Medina, Eva; Loof, Torsten G

    2016-11-01

    Coagulation is a mechanism for wound healing after injury. Several recent studies delineate an additional role of the intrinsic pathway of coagulation, also known as the contact system, in the early innate immune response against bacterial infections. In this study, we investigated the role of factor XIII (FXIII), which is activated upon coagulation induction, during Streptococcus pyogenes-mediated skin and soft tissue infections. FXIII has previously been shown to be responsible for the immobilization of bacteria within a fibrin network which may prevent systemic bacterial dissemination. In order to investigate if the FXIII-mediated entrapment of S. pyogenes also influences the disease outcome we used a murine S. pyogenes M1 skin and soft tissue infection model. Here, we demonstrate that a lack of FXIII leads to prolonged clotting times, increased signs of inflammation, and elevated bacterial dissemination. Moreover, FXIII-deficient mice show an impaired survival when compared with wildtype animals. Additionally, local reconstitution of FXIII-deficient mice with a human FXIII-concentrate (Fibrogammin(®)P) could reduce the systemic complications, suggesting a protective role for FXIII during early S. pyogenes skin infection. FXIII therefore might be a possible therapeutically application to support the early innate immune response during skin infections caused by S. pyogenes. Copyright © 2016 Elsevier GmbH. All rights reserved.

  19. The effect of different apheresis modalities on coagulation factor XIII level during antibody removal in ABO-blood type incompatible living related renal transplantation.

    Science.gov (United States)

    Hanafusa, Norio; Hamasaki, Yoshifumi; Kawarasaki, Hiroo; Kido, Ryo; Shibagaki, Yugo; Ishikawa, Akira; Enomoto, Yutaka; Fujita, Toshiro; Noiri, Eisei; Nangaku, Masaomi

    2013-10-01

    Apheresis therapy is used to remove pathogenic antibodies within the recipient blood during ABO-incompatible living related renal transplantation (LRRT). Factor XIII (FXIII) is a coagulating factor. Its deficiency reportedly engenders perioperative bleeding. This study compared apheresis modalities from the perspective of the FXIII level. Cases 1-3 were treated only with double-filtration plasmapheresis (DFPP) without (case 1) or with (cases 2 and 3) fresh frozen plasma (FFP) supplementation. Cases 4 and 5 were treated with simple plasma exchange (PEx) with FFP supplementation for the last session. Cases 1-3 showed a marked (case 1, 8.6%) or moderate (case 2, 26.2%; case 3, 28.4%) decrease in FXIII on the day before the procedure after the last apheresis session, although cases 4 (81.9%) and 5 (66.2%) did not. Case 1 experienced perioperative bleeding. The last session is usually performed the day before the surgical procedure. Therefore, FXIII elimination by DFPP might cause bleeding complications because of its slow recovery. The fact warrants that the last apheresis modality during the course might be PEx from the viewpoint of FXIII depletion.

  20. Repletion of factor XIII following cardiopulmonary bypass using a recombinant A-subunit homodimer. A preliminary report

    DEFF Research Database (Denmark)

    Levy, Jerrold H; Gill, Ravi; Nussmeier, Nancy A

    2009-01-01

    Bleeding following cardiac surgery involving cardiopulmonary bypass (CPB) remains a major concern. Coagulation factor XIII (FXIII) functions as a clot-stabilising factor by cross-linking fibrin. Low post-operative levels of FXIII correlate with increased post-operative blood loss. To evaluate...... reviewing ECGs and cardiac enzymes. No other thromboembolic events were seen. Dosing with 25-50 IU/kg rFXIII-A(2) restored levels of FXIII to pre-operative levels, with a tendency towards an overshoot in receiving 50 IU/kg. rFXIII-A(2), in doses from 11.9 IU/kg up to 50 IU/kg, was well tolerated. For post...

  1. [Condition setting for the measurement of blood coagulation factor XIII activity using a fully automated blood coagulation analyzer, COAGTRON-350].

    Science.gov (United States)

    Kanno, Nobuko; Kaneko, Makoto; Tanabe, Kumiko; Jyona, Masahiro; Yokota, Hiromitsu; Yatomi, Yutaka

    2012-12-01

    The automated laboratory analyzer COAGTRON-350 (Trinity Biotech) is used for routine and specific coagulation testing for the detection of fibrin formation utilizing either mechanical principles (ball method) or photo-optical principles, chromogenic kinetic enzyme analysis, and immune-turbidimetric detection systems in one benchtop unit. In this study, we demonstrated and established a parameter for the measurement of factor XIII (FXIII) activity using Berichrom FXIII reagent and the COAGTRON-350 analyzer. The usual protocol used for this reagent, based on the handling method, was slightly modified for this device. The analysis showed that fundamental study for the measurement of FXIII activity under our condition setting was favorable in terms of reproducibility, linearity, and correlation with another assays. Since FXIII is the key enzyme that plays important roles in hemostasis by stabilizing fibrin formation, the measurement of FXIII is essential for the diagnosis of bleeding disorders. Therefore, FXIII activity assessment as well as a routine coagulation testing can be conducted simultaneously with one instrument, which is useful in coagulopathy assessment.

  2. Congenital deficiency of factor VII.

    Science.gov (United States)

    Sikka, M; Gomber, S; Madan, N; Rusia, U; Sharma, S

    1996-01-01

    A case of congenital factor VII deficiency in a five-year-old child is reported. The patient, born of a non-consanguineous marriage, presented with repeated bouts of epistaxis since childhood. The prothrombin time (PT) was markedly prolonged with a normal bleeding time (BT), partial thromboplastin time with Kaolin (PTTK) and platelet count. The patient has been on follow up for the last four years and is doing apparently well.

  3. Factor XIII A-Subunit V34L Variant Affects Thrombus Cross-Linking in a Murine Model of Thrombosis.

    Science.gov (United States)

    Duval, Cédric; Ali, Majid; Chaudhry, Waleed W; Ridger, Victoria C; Ariëns, Robert A S; Philippou, Helen

    2016-02-01

    Factor XIII (FXIII) cross-links fibrin upon activation by thrombin. Activation involves cleavage at residue 37 by thrombin, releasing an activation peptide. A common polymorphism (valine to leucine variant at residue 34, V34L), located in the activation peptide, has been associated with increased activation rates and paradoxically a protective effect in cardiovascular disease. There is, currently, no data available on the effects of V34L from in vivo models of thrombosis. We examined the effect of FXIII V34L on clot formation and cross-linking in vivo. We generated a panel of full-length recombinant human FXIII-A2 variants with amino acid substitutions in the activation peptide to investigate the effect of these variants on activation rate, and we used wild-type, V34L, and alanine to glycine variant at residue 33 variants to study the effects of varying FXIII activation rate on thrombus formation in a murine model of FeCl3 injury. FXIII activation assay showed that residues 29, 30, 33, and 34 play a critical role in thrombin interaction. Full-length recombinant human FXIII-A2 V34L has significant effects on clot formation, structure, and lysis in vitro, using turbidity assay. This variant influenced fibrin cross-linking but not size of the thrombus in vivo. Mutations in the activation peptide of full-length recombinant FXIII regulate activation rates by thrombin, and V34L influences in vivo thrombus formation by increased cross-linking of the clot. © 2016 American Heart Association, Inc.

  4. Genetics Home Reference: factor VII deficiency

    Science.gov (United States)

    ... VII deficiency , is caused by mutations in the F7 gene, which provides instructions for making a protein ... about the gene associated with factor VII deficiency F7 Related Information What is a gene? What is ...

  5. Effect of Factor XIII-A G185T Polymorphism on Visual Prognosis after Photodynamic Therapy for Neovascular Macular Degeneration

    Directory of Open Access Journals (Sweden)

    Francesco Parmeggiani

    2015-08-01

    Full Text Available Macular degenerations represent leading causes of central blindness or low vision in developed countries. Most of these severe visual disabilities are due to age-related macular degeneration (AMD and pathologic myopia (PM, both of which are frequently complicated by subfoveal choroidal neovascularization (CNV. Photodynamic therapy with verteporfin (PDT-V is still employed for CNV treatment in selected cases or in combined regimen. In Caucasian patients, the common polymorphism G185T of factor XIII-A gene (FXIII-A-G185T; rs5985 has been described as predictor of poor angiographic CNV responsiveness to PDT-V. Nevertheless, the prognostic implications of this pharmacogenetic determinant on long-term visual outcome after a PDT-V regimen have not been evaluated. We retrospectively selected Caucasian patients presenting with treatment-naive CNV and receiving standardized PDT-V protocol for two years. The study population included patients affected by subfoveal CNV secondary to AMD or PM. We assessed the correlations between the polymorphic allele T of FXIII-A-G185T and: (1 total number of photodynamic treatments; and (2 change in visual acuity from baseline to the end of the follow-up period. Considering a total study population of 412 patients with neovascular AMD or PM, the carriers of 185 T-allele of FXIII-A (GT or TT genotype received a higher number of photodynamic treatments than patients without it (GG wild-type genotype (p < 0.01; mean number of PDT-V: 5.51 vs. 3.76, respectively. Moreover, patients with 185 T-allele of FXIII-A had a more marked worsening of visual acuity at 24 months than those with the GG-185 wild genotype (p < 0.01; mean difference in logMAR visual acuity: 0.22 vs. 0.08, respectively. The present findings show that the G185T polymorphism of the FXIII-A gene is associated with significant differences in the long-term therapeutic outcomes of patients treated with standardized PDT-V protocol. The comprehensive appraisal of

  6. Genetics Home Reference: factor XI deficiency

    Science.gov (United States)

    ... common feature of factor XI deficiency is prolonged bleeding after trauma or surgery, especially involving the inside of the mouth and ... nasal cavities ) or the urinary tract . If the bleeding is left untreated after surgery, solid swellings consisting of congealed blood (hematomas) can ...

  7. Genetic, molecular and functional analyses of complement factor I deficiency

    DEFF Research Database (Denmark)

    Nilsson, S.C.; Trouw, L.A.; Renault, N.;

    2009-01-01

    Complete deficiency of complement inhibitor factor I (FI) results in secondary complement deficiency due to uncontrolled spontaneous alternative pathway activation leading to susceptibility to infections. Current genetic examination of two patients with near complete FI deficiency and three...

  8. Epistatic and pleiotropic effects of polymorphisms in the fibrinogen and coagulation factor XIII genes on plasma fibrinogen concentration, fibrin gel structure and risk of myocardial infarction.

    Science.gov (United States)

    Mannila, Maria Nastase; Eriksson, Per; Ericsson, Carl-Göran; Hamsten, Anders; Silveira, Angela

    2006-03-01

    An intricate interplay between the genes encoding fibrinogen gamma (FGG), alpha (FGA) and beta (FGB), coagulation factor XIII (F13A1) and interleukin 6 (IL6) and environmental factors is likely to influence plasma fibrinogen concentration, fibrin clot structure and risk of myocardial infarction (MI). In the present study, the potential contribution of SNPs harboured in the fibrinogen, IL6 and F13A1 genes to these biochemical and clinical phenotypes was examined. A database and biobank based on 387 survivors of a first MI and population-based controls were used. Sixty controls were selected according to FGG 9340T > C [rs1049636] genotype for studies on fibrin clot structure using the liquid permeation method. The multifactor dimensionality reduction method was used for interaction analyses. We here report that the FGA 2224G > A [rs2070011] SNP (9.2%), plasma fibrinogen concentration (13.1%) and age (8.1%) appeared as independent determinants of fibrin gel porosity. The FGA 2224G > A SNP modulated the relation between plasma fibrinogen concentration and fibrin clot porosity. The FGG-FGA*4 haplotype, composed of the minor FGG 9340C and FGA 2224A alleles, had similar effects, supporting its reported protective role in relation to MI. Significant epistasis on plasma fibrinogen concentration was detected between the FGA 2224G > A and F13A1 Val34Leu [rs5985] SNPs (p FGG 9340T > C and FGB 1038G > A [rs1800791] SNPs appeared to interact on MI risk, explaining the association of FGG-FGB haplotypes with MI in the absence of effects of individual SNPs. Thus, epistatic and pleiotropic effects of polymorphisms contribute to the variation in plasma fibrinogen concentration, fibrin clot structure and risk of MI.

  9. Genetics Home Reference: factor X deficiency

    Science.gov (United States)

    ... deficiency occurs in approximately 1 per million individuals worldwide. Related Information What information about a genetic condition can statistics provide? Why are some genetic conditions more common ...

  10. Familial growth hormone releasing factor deficiency in pseudopseudohypoparathyroidism.

    OpenAIRE

    Stirling, H F; Barr, D G; Kelnar, C J

    1991-01-01

    A mother with pseudopseudohypoparathyroidism and her short son showed poor spontaneous growth hormone secretion, and provocation tests suggested a deficiency of growth hormone releasing factor. This is the first report of growth hormone releasing factor deficiency in pseudopseudohypoparathyroidism. The boy has responded well to growth hormone treatment over a period of three years.

  11. Risk factors associated with anemia, iron deficiency and iron deficiency anemia in rural Nepali pregnant women.

    Science.gov (United States)

    Makhoul, Zeina; Taren, Douglas; Duncan, Burris; Pandey, Pooja; Thomson, Cynthia; Winzerling, Joy; Muramoto, Myra; Shrestha, Ram

    2012-05-01

    We conducted a cross sectional study to investigate risk factors associated with severe anemia [hemoglobin (Hb) iron status among Nepali pregnant women. Socio-demographic, anthropometric, health and dietary data were collected from 3,531 women living in the southeastern plains of Nepal. Stool samples were analyzed for intestinal helminthes. Dark adaptation was assessed using the Night Vision Threshold Test (NVTT). Hb levels were measured in all subjects to detect anemia and the soluble transferrin receptor (sTfR) was measured among a subsample of 479 women. The iron status categories were: 1) normal (Hb> or = 11.0 g/dl and sTfR anemia without iron deficiency (Hbiron deficiency without anemia (Hb > or = 11.0 g/dl and sTfR>8.5 mg/l); and 4) iron deficiency anemia (IDA): (Hb8.5 mg/l). Factors associated with severe anemia and poor iron status were determined using logistic regression. Hookworm infection increased the risk for developing severe anemia [adjusted odds ratio (AOR): 4.26; 95% CI 1.67-10.89; piron deficiency with and without anemia. Intake of iron supplements as tablets and/or tonic was protective against severe anemia, anemia without iron deficiency and IDA. Dietary heme iron was significantly associated with iron deficiency without anemia (RRR: 0.1; 95% CI 0.02-0.47; panemia and associated nutrient deficiencies.

  12. Evaluation of factor IX deficiency by interdigitated electrode (IDE)

    Science.gov (United States)

    Gopinath, Subash C. B.; Hashim, Uda; Uda, M. N. A.

    2017-03-01

    Factor IX deficiency is the main cause of hemophilia A and B. This a severe excessive bleeding disorder that can even kill the patient if not treated with the right prescription of Factor IX hormone to stop the bleeding. The bleeding can be caused by an injury or even a sudden bleeding in some very rare cases. To find the Factor IX effectiveness and to understand the deficiency more carefully for the future of medicine, experiments are conducted to test the Factor IX using the Interdigitated Electrode (IDE) and gold Nanoparticle with the help of Nanoelectrical technology.

  13. Classical factor X deficiency. Report of a further case.

    Science.gov (United States)

    Girolami, A; Coser, P; Brunetti, A; Prinoth, O

    1975-01-01

    A case of classical factor X deficiency is reported. The propositus is a 28-year-old male who presented easy bruising, epistaxis, hematomas, hematuria and occasional hemartrosis since early childhood. The severely prolonged prothrombin time was corrected by normal serum but not by adsorbed normal plasma. The abnormality was not corrected by the plasma of a patient with factor X deficiency, but by the plasma of patients with factor II or VII deficiencies. Partial thromboplastin time, prothrombin consumption and the thromboplastin generation test were abnormal. The thromboelastogram showed a prolonged 'K' and 'r' together with a normal 'ma'. Factor X was very low (smaller than 1%). Platelet tests were normal. No factor X band or precipitates were seen on electroimmunoassay and on the cross-over electrophoresis. The non-consanguineous parents and several other members of the family were found to be heterozygotes.

  14. Factor XI deficiency diagnosed following use of adalimumab

    Directory of Open Access Journals (Sweden)

    Guven Cetin

    2014-01-01

    Full Text Available Adalimumab is a drug used in the treatment of refractory psoriasis. We present a case of a 55-year-old male patient who developed petechiae and purpura after the ninth dose of adalimumab therapy. The results of laboratory investigations revealed factor XI (F.XI deficiency. It should be recognized that F XI deficiency may develop in patients using long-term adalimumab, leading to increased risk of bleeding.

  15. Prevalence and risk factors of iodine deficiency among schoolchildren.

    Science.gov (United States)

    Gür, Emel; Ercan, Oya; Can, Günay; Akkuş, Semra; Güzelöz, Sima; Ciftcili, Serdar; Arvas, Ahmet; Iltera, Ozdemir

    2003-06-01

    The aim of this study was to demonstrate the prevalence of iodine deficiency among schoolchildren and the risk factors influencing it. One thousand five hundred and seventy-three schoolchildren were chosen from 14 schools in seven different regions in Istanbul. After all data relating to sociodemographic factors and the use of iodized salt were recorded, iodine contents of urine samples were determined by the Sandell Kolthoff reaction. Chi-squared and multiple regression analysis were used for the investigation of the correlation between iodine deficiency and risk factors. The prevalence of goitre (> or = 2 grade) was 1.9 per cent. The median urine iodine level was in the normal range (10.5 microg/dl). In 46.2 per cent of the students the urine iodine levels were below 10 microg/dl and 13.9 per cent of the students had urine iodine levels below 5 microg/dl. The prevalence of iodine deficiency was significantly higher in younger (iodine deficiency was significantly higher in females than in males and also higher in children who lived on the European side of Istanbul than on the Anatolian side of Istanbul (p iodine deficiency rate was not affected by the use of iodized salt (p > 0.05). Although the median urine iodine level was in the normal range, in 46.2 per cent of the students urine iodine levels were low (iodine deficiency, the use of a more stable potassium iodate for the fortification of salt may be required.

  16. Refractory Epistaxis due to Severe Factor V Deficiency with Inhibitor

    Directory of Open Access Journals (Sweden)

    Elizabeth S. John

    2015-01-01

    Full Text Available Factor V deficiency secondary to inhibitors is extremely rare and can be caused by a wide collection of exposures such as bovine thrombin and beta lactamase antibiotics. The management of factor V deficiency with inhibitor is a condition treated based on case reports due to the rarity of this condition. We describe a complicated case of an elderly patient with severe factor V deficiency with high inhibitor titer refractory to FEIBA (anti-inhibitor coagulation complex treated with NovoSeven concurrently with cyclosporine immunosuppression and Rituxan. Given that there are no consensus guidelines on treatment, this case offers important insight into the therapeutic approaches that can be used to treat such patients.

  17. Rapid detection of the factor XIII Val34Leu (163 G-->T) polymorphism by real-time PCR using fluorescence resonance energy transfer detection and melting curve analysis.

    Science.gov (United States)

    Shemirani, Amir H; Muszbek, László

    2004-01-01

    The Val34Leu polymorphism in the A subunit of blood coagulation factor XIII (FXIII-A) is located in the activation peptide, just three amino acids upstream of the thrombin cleavage site. The Val-->Leu replacement accelerates the rate of the proteolytic activation of FXIII and it seems to provide protection against myocardial infarction. Methods available for the assessment of the FXIII-A Val34Leu polymorphism are rather time-consuming, laborious and not easily applicable for large-scale studies. In this study a new method based on real-time PCR with fluorescence resonance energy transfer (FRET) detection and melting curve analysis was developed. The rapid, simple method was adapted to the widely used real-time PCR instrument, LightCycler (Roche Diagnostics). The results showed 100% coincidence with those obtained by the traditional PCR-restriction fragment length polymorphism (RFLP) assay and fluorescent DNA sequencing. Using this method, an allele frequency of 24.2% was obtained (n=113), which well agrees with the allele frequency obtained by PCR-RFLP on a different group of the same ethnic Hungarian population (25.9%).

  18. 21 CFR 864.7290 - Factor deficiency test.

    Science.gov (United States)

    2010-04-01

    ... defects, to monitor certain types of therapy, to detect coagulation inhibitors, and to detect a carrier state (a person carrying both a recessive gene for a coagulation factor deficiency such as hemophilia and the corresponding normal gene). (b) Classification. Class II (performance standards). [45 FR 60613...

  19. A case of factor X deficiency in a Chihuahua dog.

    Science.gov (United States)

    Heuss, Jessica; Weatherton, Linda

    2016-08-01

    A juvenile Chihuahua dog developed hemoperitoneum after routine ovariohysterectomy. She was managed with packed red blood cell and fresh frozen plasma transfusions as well as an exploratory laparotomy to verify ligature sites. No recurrence of hemorrhage occurred. Factor X deficiency was diagnosed and confirmed with repeat analysis including during times of health.

  20. Constructive proof of deficiency theorem of (g,f)-factor

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Berge(1958) gave a formula for computing the deficiency of maximum matchings of a graph.More generally,Lov’asz obtained a deficiency formula of(g,f)-optimal graphs and consequently a criterion for the existence of(g,f)-factors.Moreover,Lov’asz proved that there is one of these decompositions which is "canonical" in a sense.In this paper,we present a short constructive proof for the defficiency formula of(g,f)-optimal graphs,and the proof implies an efficient algorithm of time complexity O(g(V)|E|) for computing the deficiency.Furthermore,this proof implies this canonical decomposition via efficient algorithms(i.e.,in polynomial time).

  1. Carnitine deficiency: Risk factors and incidence in children with epilepsy.

    Science.gov (United States)

    Fukuda, Mitsumasa; Kawabe, Mika; Takehara, Makoto; Iwano, Sachiko; Kuwabara, Kozue; Kikuchi, Chiya; Wakamoto, Hiroyuki; Morimoto, Takehiko; Suzuki, Yuka; Ishii, Eiichi

    2015-09-01

    Carnitine deficiency is relatively common in epilepsy; risk factors reportedly include combination antiepileptic drug (AED) therapy with valproic acid (VPA), young age, intellectual disability, diet and enteral or parenteral feeding. Few studies have examined the correlation between each risk factor and carnitine deficiency in children with epilepsy. We examined the influence of these risk factors on carnitine deficiency, and identified a formula to estimate plasma free carnitine concentration in children with epilepsy. Sixty-five children with epilepsy and 26 age-matched controls were enrolled. Plasma carnitine concentrations were measured using an enzyme cycling assay, and correlations were sought with patients' other clinical characteristics. Carnitine deficiency was found in approximately 17% of patients with epilepsy and was significantly associated with carnitine-free enteral formula only by tube feeding, number of AEDs taken (independent of VPA use), body weight (BW), body height and Gross Motor Function Classification System (GMFCS) score. Stepwise multiple linear regression analysis indicated that carnitine concentration (in μmol/L) could be accurately estimated from a formula that does not require blood testing: 42.44+0.14×(BW in kg)-18.16×(feeding)-3.19×(number of AEDs), where feeding was allocated a score of 1 for carnitine-free enteral formula only by tube feeding and 0 for taking food orally (R(2)=0.504, Pepilepsy. l-carnitine should be administered to children at risk of deficiency to avoid complications. Treatment decisions can be informed using an estimation formula that does not require blood tests. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  2. CRM+ severe Fletcher factor deficiency associated with Graves' disease.

    Science.gov (United States)

    Kyrle, P A; Niessner, H; Deutsch, E; Lechner, K; Korninger, C; Mannhalter, C

    1984-01-01

    A 59-year-old male patient with Graves' disease and severe hereditary Fletcher factor deficiency is described. PKK clotting activity as well as the activity by a chromogenic substrate method (Chromozym PK) was less then 0.01 U/ml. In contrast to functional tests, the immunological assay (Laurell method) showed a PKK antigen concentration of 0.25 U/ml, indicating the presence of an abnormal nonfunctional PKK molecule (CRM+ variant). An inhibitor was excluded since the patient plasma did not inactivate partially purified PKK. Investigation of 11 family members revealed a reduction of the PKK clotting activity in 9 relatives of the patient. Since Graves' disease is considered an autoimmune disease, our case represents an example of an association of a severe hereditary deficiency of a contact factor and an autoimmune disease.

  3. Vena porta thrombosis in patient with inherited factor VII deficiency

    DEFF Research Database (Denmark)

    Klovaite, Jolanta; Friis-Hansen, Lennart Jan; Larsen, Fin S;

    2010-01-01

    with inherited FVII deficiency and chronic vena porta thrombosis. She presented at 32 weeks of gestation with spontaneously increased international normalized ratio, severe thrombocytopenia and very few unspecific symptoms. The extensive examination of the patient revealed cavernous transformation of the portal...... vein with well expressed portosystemic collaterals, heterozygosity for three common polymorphisms in FVII gene, associated with reduction in plasma FVII levels, and no other factors predisposing to thrombosis....

  4. Deficiencies

    Data.gov (United States)

    U.S. Department of Health & Human Services — A list of all deficiencies currently listed on Nursing Home Compare, including the nursing home that received the deficiency, the associated inspection date,...

  5. An immunologic approach to induction of epidermal growth factor deficiency

    DEFF Research Database (Denmark)

    Raaberg, Lasse; Nexø, Ebba; Poulsen, Steen Seier;

    1995-01-01

    Epidermal growth factor (EGF) in pharmacologic doses is able to induce growth and development in the fetus and the newborn. To investigate the opposite situation, the effects of insufficient amounts of EGF during development, we wanted to establish an in vivo model with a state of EGF deficiency......(-9) mol/L. The antibodies recognized purified EGF from the submandibular glands (6 kD) and from urine (45 kD) and further native EGF in saliva and urine. The cross-reactivity toward transforming growth factor-alpha was below 3%. Binding of EGF by antibodies inhibited its binding to the EGF...

  6. Secondary failure of plasma therapy in factor H deficiency.

    Science.gov (United States)

    Nathanson, Sylvie; Ulinski, Tim; Frémeaux-Bacchi, Véronique; Deschênes, Georges

    2006-11-01

    We report a patient with homozygous factor H deficiency leading to permanent alternate complement activation and early onset of the hemolytic uremic syndrome. He was successfully treated with weekly infusions of fresh frozen plasma over 4 years, displaying normal blood pressure while only treated with an angiotensin converting enzyme (ACE) inhibitor, a steady level of haptoglobin, low-range proteinuria and normal creatinine clearance. By the end of the fourth year of treatment, he dramatically developed a relapse of hemolytic and uremic syndrome, displaying undetectable haptoglobin, nephrotic range proteinuria and progressive renal failure. Despite a ten-fold increase in the dosage of plasma infusion through daily plasma exchange, haptoglobin remained undetectable while circulating antigenic factor H levels reached 22-24% (normal values 65-140%). Three months following the biological onset of the relapse, a bilateral nephrectomy was performed owing to uncontrolled hypertension and rapidly progressive renal failure. The molecular mechanism of plasma resistance remained unclear while antifactor H antibodies were not detected in the plasma. We suggest that protracted administration of exogenous factor H might not be a long-term strategy in homozygous factor H deficiency.

  7. Insulin-Like Growth Factor-1 Deficiency and Cirrhosis Establishment

    Science.gov (United States)

    de la Garza, Rocio G.; Morales-Garza, Luis Alonso; Martin-Estal, Irene; Castilla-Cortazar, Inma

    2017-01-01

    Cirrhosis represents the final stage of chronic liver damage, which can be due to different factors such as alcohol, metabolic syndrome with liver steatosis, autoimmune diseases, drugs, toxins, and viral infection, among others. Nowadays, cirrhosis is an important health problem and it is an increasing cause of morbidity and mortality, being the 14th most common cause of death worldwide. The physiopathological pathways that lead to fibrosis and finally cirrhosis partly depend on the etiology. Nevertheless, some common features are shared in this complex mechanism. Recently, it has been demonstrated that cirrhosis is a dynamic process that can be altered in order to delay or revert fibrosis. In addition, when cirrhosis has been established, insulin-like growth factor-1 (IGF-1) deficiency or reduced availability is a common condition, independently of the etiology of chronic liver damage that leads to cirrhosis. IGF-1 deprivation seriously contributes to the progressive malnutrition of cirrhotic patient, increasing the vulnerability of the liver to establish an inflammatory and oxidative microenvironment with mitochondrial dysfunction. In this context, IGF-1 deficiency in cirrhotic patients can justify some of the common characteristics of these individuals. Several studies in animals and humans have been done in order to test the replacement of IGF-1 as a possible therapeutic option, with promising results. PMID:28270882

  8. Prevalence of Vitamin D Deficiency and Its Related Factors Among University Students in Shiraz, Iran

    Directory of Open Access Journals (Sweden)

    Shiva Faghih

    2014-01-01

    Conclusions: Vitamin D deficiency especially among female students is alarmingly prevalent. Increasing use of sunscreen lotion and clothing style could be the main factors inhibiting endogenous vitamin D synthesis which results in its deficiency.

  9. Comprehensive Behavioral Analysis of Activating Transcription Factor 5-Deficient Mice.

    Science.gov (United States)

    Umemura, Mariko; Ogura, Tae; Matsuzaki, Ayako; Nakano, Haruo; Takao, Keizo; Miyakawa, Tsuyoshi; Takahashi, Yuji

    2017-01-01

    Activating transcription factor 5 (ATF5) is a member of the CREB/ATF family of basic leucine zipper transcription factors. We previously reported that ATF5-deficient (ATF5(-/-)) mice demonstrated abnormal olfactory bulb development due to impaired interneuron supply. Furthermore, ATF5(-/-) mice were less aggressive than ATF5(+/+) mice. Although ATF5 is widely expressed in the brain, and involved in the regulation of proliferation and development of neurons, the physiological role of ATF5 in the higher brain remains unknown. Our objective was to investigate the physiological role of ATF5 in the higher brain. We performed a comprehensive behavioral analysis using ATF5(-/-) mice and wild type littermates. ATF5(-/-) mice exhibited abnormal locomotor activity in the open field test. They also exhibited abnormal anxiety-like behavior in the light/dark transition test and open field test. Furthermore, ATF5(-/-) mice displayed reduced social interaction in the Crawley's social interaction test and increased pain sensitivity in the hot plate test compared with wild type. Finally, behavioral flexibility was reduced in the T-maze test in ATF5(-/-) mice compared with wild type. In addition, we demonstrated that ATF5(-/-) mice display disturbances of monoamine neurotransmitter levels in several brain regions. These results indicate that ATF5 deficiency elicits abnormal behaviors and the disturbance of monoamine neurotransmitter levels in the brain. The behavioral abnormalities of ATF5(-/-) mice may be due to the disturbance of monoamine levels. Taken together, these findings suggest that ATF5(-/-) mice may be a unique animal model of some psychiatric disorders.

  10. Factor VII deficiency impairs cutaneous wound healing in mice.

    Science.gov (United States)

    Xu, Zhi; Xu, Haifeng; Ploplis, Victoria A; Castellino, Francis J

    2010-01-01

    Skin keratinocytes express tissue factor (TF) and are highly associated with skin wound healing. Although it has been demonstrated that perivascular TF expression in granulation tissue formed after dermal injury is downregulated during healing, studies of the mechanism of factor (F) VII, a TF ligand, in skin wound healing are lacking. We reported the use of a dermal punch model to demonstrate that low-expressing FVII mice (approximately 1% of wild type [WT]) exhibited impaired skin wound healing compared with WT controls. These low-FVII mice showed defective reepithelialization and reduced inflammatory cell infiltration at wound sites. This attenuated reepithelialization was associated with diminished expression of the transcription factor early growth response 1 (Egr-1). In vitro, Egr-1 was shown to be essential for the FVIIa-induced regulation of keratinocyte migration and inflammation. Both Egr-1 upregulation and downstream inflammatory cytokine appearance in keratinocytes depended on FVIIa/TF/protease-activated receptor 2 (PAR-2)-induced signaling and did not require subsequent generation of FXa and thrombin. The participation of Egr-1 in FVIIa-mediated regulation of keratinocyte function was confirmed by use of Egr-1-deficient mice, wherein a significant delay in skin wound healing after injury was observed, relative to WT mice. The results from these studies demonstrate an in vivo mechanistic relationship between FVIIa, Egr-1 and the inflammatory response in keratinocyte function during the wound healing process.

  11. Iodine deficiency and associated factors among school children: a cross-sectional study in Ethiopia

    OpenAIRE

    Sintayehu Hailu; Mamo Wubshet; Haile Woldie; Amare Tariku

    2016-01-01

    Abstract Background Iodine deficiency remains a public health problem in the world. It is the leading cause of preventable mental retardation and brain damage worldwide. Though 12 million school age children are at risk of developing iodine deficiency, there is a scarcity of literature showing the magnitude of iodine deficiency in Ethiopia. Therefore, this study aimed to determine the prevalence and associated factors of iodine deficiency among school children in Robe District, southeast Ethi...

  12. An accompanying genetic severe deficiency of tissue factor protects mice with a protein C deficiency from lethal endotoxemia.

    Science.gov (United States)

    Castellino, Francis J; Donahue, Deborah L; Navari, Rudolph M; Ploplis, Victoria A; Walsh, Mark

    2011-01-06

    Mice with a severe genetic deficiency of protein C (PC), PC(-/-)PC(tg4), display enhanced susceptibility to lethal effects of gram-negative endotoxemia induced by lipopolysaccharide (LPS), whereas mice severely deficient in tissue factor (TF), TF(-/-)hTF(tg), are protected from LPS-mediated lethality. In this study, we show that a simultaneous severe deficiency of TF protected low-PC mice from LPS-induced death, resulting in a survival profile similar to that experienced by wild-type (WT) mice. Plasma and whole blood coagulation assays, the latter measured by thromboelastography, demonstrated development of coagulopathies in LPS-treated mice, which were more severe in the case of the doubly deficient TF(-/-)hTF(tg)/PC(-/-)PC(tg4) mice, mainly reflecting earlier signs of disseminated intravascular coagulation in this latter cohort. Markers of inflammation were also elevated in response to LPS in both groups of mice at times just preceding death. We conclude that whereas coagulopathies are more exacerbated in LPS-treated TF(-/-)hTF(tg)/PC(-/-)PC(tg4) mice, the lowering of TF levels in mice with an accompanying severe PC deficiency confers protection against death compared with mice with a single severe PC deficiency. This suggests that proteases generated as a result of factor VIIa/TF-mediated thrombin generation play a mechanistic role in the enhanced lethality seen under very low PC conditions in an endotoxemia model in mice.

  13. Coagulation factor deficiency apparently related to the Fitzgerald trait: the first cases in Japan.

    Directory of Open Access Journals (Sweden)

    Hayashi,Hisamoto

    1978-04-01

    Full Text Available A blood coagulation deficiency was found at the contact phase in identical Japanese female twins. Of the four possible factors involved, Factor XI or XII can be ruled out according to cross-correction studies. The problem factor was probably not Fletcher factor, because the abnormal partial thromboplastin time was not significantly shortened by increasing the incubation period of plasma with kaolin. The deficiency is most likely due to the lack of Fitzgerald factor.

  14. piggyBac-mediated phenotypic correction of factor VIII deficiency

    Directory of Open Access Journals (Sweden)

    Janice M Staber

    2014-01-01

    Full Text Available Hemophilia A, caused by a deficiency in factor VIII (FVIII, is the most severe inherited bleeding disorder. Hemophilia A is an attractive gene therapy candidate because even small increases in FVIII levels will positively alter the phenotype. While several vectors are under investigation, gene addition from an integrated transgene offers the possibility of long term expression. We engineered the DNA transposon-based vector, piggyBac (PB, to carry a codon-optimized B-domain deleted human FVIII cDNA. Evaluation of gene transfer efficiency in FVIII null mice demonstrated that PB containing the FVIII cDNA, delivered via hydrodynamic injection to immunocompetent hemophilia mice, conferred persistent gene expression, attaining mean FVIII activity of approximately 60% with 3/19 developing inhibitors. In addition to efficacious expression, a goal of gene transfer-based therapies is to develop vectors with low toxicity. To assess endoplasmic reticulum stress in hepatocytes stably expressing the transgene, we evaluated levels of ER stress markers via qPCR and found no evidence of cell stress. To evaluate phenotypic correction, a tail clip assay performed at the end of the study revealed reduced blood loss. These data demonstrate that PB can be used to achieve sustained FVIII expression and long-term therapeutic benefit in a mouse model.

  15. Rosai-Dorfman disease with factor XII deficiency.

    Science.gov (United States)

    Kasapoglu Gunal, Esen; Kamali, Sevil; Akdogan, Mehmet Fatih; Cimen, Arif Oguz; Ocal, Lale; Agan, Mehmet; Gul, Ahmet; Inanc, Murat; Konice, Meral; Aral, Orhan

    2009-06-01

    A 17-year-old female patient presented with chronic symmetrical oligoarthritis of both knees and ankles, xerostomia, xerophthalmia, multiple bilateral lymphadenopathies in the cervical region, and bilateral parotid enlargement with the histological finding of chronic sialoadenitis. She had been already given methotrexate, chloroquine, and corticosteroids with the diagnosis of rheumatoid arthritis (RA) before referral to our outpatient clinic. Because her complaints and the lumps did not remit and she could be classified as neither RA nor primary Sjögren's syndrome (SS) according to 1987 ACR RA criteria or European preliminary criteria for SS, lymph node biopsy was repeated and revealed the diagnosis of Rosai-Dorfman disease (RDD) with the histological findings of histiocytes, phagocyting lymphocytes in enlarged sinuses, and mature plasma cells infiltrating the pulpa. All the medications were stopped after the pathological diagnosis of RDD and consulting with the Division of Hematology. She was reevaluated with magnetic resonance imaging, which showed dense infiltrative areas around knee and ankle joints, and computed tomography that showed a soft tissue mass surrounding the descending aorta and upper part of the abdominal aorta. Activated partial thromboplastin time was found to be prolonged in prebiopsy examinations, and factor XII deficiency was detected after detailed hematological evaluation. The symptoms of joint involvement were relieved with nonsteroidal antiinflammatory drugs. She has been followed-up without medication without obvious clinical or laboratory change. We herein report a patient with RDD mimicking RA and SS. We consider that RDD should be kept in mind especially in patients with resistant symptoms to conventional therapies, younger disease onset, and predominant parotid and lymph node enlargement.

  16. Anaesthesia management of caesarean section in a patient with severe factor XI deficiency

    Directory of Open Access Journals (Sweden)

    Debesh Bhoi

    2013-01-01

    Full Text Available Factor XI deficiency is a rare coagulation disorder associated with bleeding tendency and prolonged APTT. Parturients can have increased bleeding during vaginal delivery or cesarean section. Patients with severe factor XI deficiency should receive prophylactic fresh frozen plasma or factor XI transfusion in the peripartum period to maintain a near normal APTT. Limited evidence based on case reports and series is inconclusive as to the choice of anesthesia technique for cesarean section. We describe the anesthesia management of a parturient with severe factor XI deficiency for cesarean section and discuss the relevant literature.

  17. Vitamin D deficiency in Crohn's disease: prevalence, risk factors and supplement use in an outpatient setting.

    LENUS (Irish Health Repository)

    Suibhne, Treasa Nic

    2012-03-01

    Vitamin D deficiency impacts on bone health and has potential new roles in inflammation. We aimed to determine the prevalence of and risk factors for vitamin D deficiency and to explore vitamin D supplement usage in patients with Crohn\\'s disease (CD) in an outpatient setting, compared with controls.

  18. Gynaecological and obstetrical bleeding in women with factor XI deficiency - a systematic review

    NARCIS (Netherlands)

    Wiewel-Verschueren, S.; Arendz, I. J.; Knol, H. M.; Meijer, Karina

    Menstrual bleeding, pregnancy and delivery present an intrinsic haemostatic challenge to women with bleeding disorders such as factor XI (FXI) deficiency. Aim: To provide a systematic overview of studies on gynaecological and obstetrical bleeding problems in women with FXI deficiency. Methods: We

  19. What factors contribute to successful appeals of nursing homes’ deficiencies in the informal dispute resolution process?

    Science.gov (United States)

    Li, Yue; Weimer, David L.; Spector, William D.; Bailey, Lauren; Harrington, Charlene

    2012-01-01

    Objectives To determine what factors contribute to successful appeals of nursing home deficiencies in the Informal Dispute Resolution (IDR) process. Design We merged CMS data about IDRs with OSCAR data about nursing home characteristics. We performed multivariate statistical analyses to predict successful appeals as a function of characteristics of the deficiency being appealed, the survey that triggered the deficiency, characteristics of the nursing home, and the state. Setting All nursing homes nationally in the period 2005–2008. Measurements Successful appeals were defined as those in which the deficiency was removed or its severity or scope reduced. Independent variables included the CMS measures of severity and scope of deficiency, abuse and neglect, substandard care, total number of deficiencies in the survey, whether the IDR was triggered by a survey or complaint, facility ownership and reputation, and state stringency of regulation. Results 26% of submitted IDRs were successful in 2005–2008. Success was more likely for less severe deficiencies, when deficiencies were triggered by a survey rather than a complaint, and when fewer deficiencies were included in the appeal. Facility ownership and state stringency of regulation were not significantly associated with the IDR success. Conclusions Overall, 2.6% of deficiencies issued were overturned through the IDR process. Further study is required to determine the appropriateness of these overturned cases and the opportunities they offer to improve the survey process. PMID:23141210

  20. Obesity as an Emerging Risk Factor for Iron Deficiency

    Directory of Open Access Journals (Sweden)

    Elmar Aigner

    2014-09-01

    Full Text Available Iron homeostasis is affected by obesity and obesity-related insulin resistance in a many-facetted fashion. On one hand, iron deficiency and anemia are frequent findings in subjects with progressed stages of obesity. This phenomenon has been well studied in obese adolescents, women and subjects undergoing bariatric surgery. On the other hand, hyperferritinemia with normal or mildly elevated transferrin saturation is observed in approximately one-third of patients with metabolic syndrome (MetS or nonalcoholic fatty liver disease (NAFLD. This constellation has been named the “dysmetabolic iron overload syndrome (DIOS”. Both elevated body iron stores and iron deficiency are detrimental to health and to the course of obesity-related conditions. Iron deficiency and anemia may impair mitochondrial and cellular energy homeostasis and further increase inactivity and fatigue of obese subjects. Obesity-associated inflammation is tightly linked to iron deficiency and involves impaired duodenal iron absorption associated with low expression of duodenal ferroportin (FPN along with elevated hepcidin concentrations. This review summarizes the current understanding of the dysregulation of iron homeostasis in obesity.

  1. Iron deficiency: an overlooked predisposing factor in angular cheilitis.

    Science.gov (United States)

    Murphy, N C; Bissada, N F

    1979-10-01

    Clinicians who recommend the use of antifungal agents for angular cheilitis may be treating the symptoms and not the predisposing cause of the disease. Iron deficiency should be considered as part of the differential diagnosis whenever angular cheilitis is encountered, especially in women of child-bearing age.

  2. Risk factors for vitamin D deficiency among veterans with and without HIV infection.

    Directory of Open Access Journals (Sweden)

    Alicia I Hidron

    Full Text Available We aimed to describe and compare the prevalence of vitamin D deficiency between HIV-negative and HIV-infected veterans in the southern United States, and to determine risk factors for vitamin D deficiency for HIV infected patients.Cross-sectional, retrospective study including all patients followed at the Atlanta VA Medical Center with the first 25-hydroxyvitamin D [25(OHD] level determined between January 2007 and August 2010. Multivariate logistic regression analysis was used to determine risk factors associated with vitamin D deficiency (< 20 ng/ml.There was higher prevalence of 25(OHD deficiency among HIV-positive compared to HIV-negative patients (53.2 vs. 38.5%, p <0.001. Independent risk factors for vitamin D deficiency in HIV + patients included black race (OR 3.24, 95% CI 2.28-4.60, winter season (OR 1.39, 95% CI 1.05-1.84 and higher GFR (OR 1.01, CI 1.00-1.01; increasing age (OR 0.98, 95% CI 0.95-0.98, and tenofovir use (OR 0.72, 95% CI 0.54-0.96 were associated with less vitamin D deficiency.Vitamin D deficiency is a prevalent problem that varies inversely with age and affects HIV-infected patients more than other veterans in care. In addition to age, tenofovir and kidney disease seem to confer a protective effect from vitamin D deficiency in HIV-positive patients.

  3. Effects of cellular iron deficiency on the formation of vascular endothelial growth factor and angiogenesis. Iron deficiency and angiogenesis

    Directory of Open Access Journals (Sweden)

    Eckard Jonathan

    2010-08-01

    Full Text Available Abstract Background Young women diagnosed with breast cancer are known to have a higher mortality rate from the disease than older patients. Specific risk factors leading to this poorer outcome have not been identified. In the present study, we hypothesized that iron deficiency, a common ailment in young women, contributes to the poor outcome by promoting the hypoxia inducible factor-1α (HIF-1α and vascular endothelial growth factor (VEGF formation. This hypothesis was tested in an in vitro cell culture model system. Results Human breast cancer MDA-MB-231 cells were transfected with transferrin receptor-1 (TfR1 shRNA to constitutively impair iron uptake. Cellular iron status was determined by a set of iron proteins and angiogenesis was evaluated by levels of VEGF in cells as well as by a mouse xenograft model. Significant decreases in ferritin with concomitant increases in VEGF were observed in TfR1 knockdown MDA-MB-231 cells when compared to the parental cells. TfR1 shRNA transfectants also evoked a stronger angiogenic response after the cells were injected subcutaneously into nude mice. The molecular mechanism appears that cellular iron deficiency elevates VEGF formation by stabilizing HIF-1α. This mechanism is also true in human breast cancer MCF-7 and liver cancer HepG2 cells. Conclusions Cellular iron deficiency increased HIF-1α, VEGF, and angiogenesis, suggesting that systemic iron deficiency might play an important part in the tumor angiogenesis and recurrence in this young age group of breast cancer patients.

  4. Relationship between Acquired Deficiency of Vitamin K-dependent Clotting Factors And Hemorrhage

    Institute of Scientific and Technical Information of China (English)

    杨锐; 张小平; 魏文宁; 洪梅; 杨焰; 胡豫

    2010-01-01

    This study examined the changes of activities of vitamin K-dependent clotting factors(VKDCF) under various pathological conditions and explored the relationship between acquired deficiency of VKDCFs and hemorrhage.Clinical data of 35 patients who were diagnosed as having acquired deficiency of VKDCF were retrospectively analyzed.Coagulation factors involved in the intrinsic and extrinsic pathways were detected in these patients and 41 control subjects.The results showed that the average activities of VKDCFs...

  5. Risk Factors for Postoperative Fibrinogen Deficiency after Surgical Removal of Intracranial Tumors.

    Science.gov (United States)

    Wei, Naili; Jia, Yanfei; Wang, Xiu; Zhang, Yinian; Yuan, Guoqiang; Zhao, Baotian; Wang, Yao; Zhang, Kai; Zhang, Xinding; Pan, Yawen; Zhang, Jianguo

    2015-01-01

    Higher levels of fibrinogen, a critical element in hemostasis, are associated with increased postoperative survival rates, especially for patients with massive operative blood loss. Fibrinogen deficiency after surgical management of intracranial tumors may result in postoperative intracranial bleeding and severely worsen patient outcomes. However, no previous studies have systematically identified factors associated with postoperative fibrinogen deficiency. In this study, we retrospectively analyzed data from patients who underwent surgical removal of intracranial tumors in Beijing Tiantan Hospital date from 1/1/2013to12/31/2013. The present study found that patients with postoperative fibrinogen deficiency experienced more operative blood loss and a higher rate of postoperative intracranial hematoma, and they were given more blood transfusions, more plasma transfusions, and were administered larger doses of hemocoagulase compared with patients without postoperative fibrinogen deficiency. Likewise, patients with postoperative fibrinogen deficiency had poorer extended Glasgow Outcome Scale (GOSe), longer hospital stays, and greater hospital expenses than patients without postoperative fibrinogen deficiency. Further, we assessed a comprehensive set of risk factors associated with postoperative fibrinogen deficiency via multiple linear regression. We found that body mass index (BMI), the occurrence of postoperative intracranial hematoma, and administration of hemocoagulasewere positively associated with preoperative-to-postoperative plasma fibrinogen consumption; presenting with a malignant tumor was negatively associated with fibrinogen consumption. Contrary to what might be expected, intraoperative blood loss, the need for blood transfusion, and the need for plasma transfusion were not associated with plasma fibrinogen consumption. Considering our findings together, we concluded that postoperative fibrinogen deficiency is closely associated with postoperative

  6. A Confounding Case of Inherited Factor V Deficiency Complicated by Inhibitors at First Presentation.

    Science.gov (United States)

    Subramanian, Hema; Kar, Rakhee; Charles, Deepak; Babu, Hitha; Ambika, Pagadalu; Dutta, Tarun Kumar

    2017-04-01

    Inherited factor V deficiency / Owren's disease has varied clinical manifestations ranging from asymptomatic laboratory abnormalities to massive hemorrhage. The acquired form due to inhibitors following antibiotic therapy, infection, or surgery is less common, and spontaneous development of inhibitors is not known. An 18-year-old boy presented with bleeding axillary and groin ulcers. At the age of 15, due to recurrent epistaxis and gum bleed, he was diagnosed with acquired factor V deficiency with positive inhibitor screen and treated with fresh frozen plasma (FFP) transfusion and temporary azathioprine. Coagulation workup at his current presentation also revealed acquired factor V deficiency with presence of inhibitors. The tests were repeated after 6 weeks of intermittent FFP transfusion, and the differences observed included negative inhibitor screen and complete correction on mixing studies, but factor V level was 2%. Evidence of inhibitors at presentation favored acquired disease. However, younger age of onset, detection of inhibitors 1 year after first episode of self-regressing bleed, lack of identifiable triggers, and persistent bleeding with reduced factor levels after disappearance of inhibitors favored inherited factor V deficiency. In this case report, we have described an interesting case of severe inherited factor V deficiency with spontaneous appearance and disappearance of inhibitors exhibiting nonspecific factor inhibitory activity.

  7. Prevalence and risk factors of vitamin D deficiency in patients with widespread musculoskeletal pain

    Directory of Open Access Journals (Sweden)

    Muharrem Çidem

    2013-12-01

    Full Text Available Objective: Vitamin D deficiency is a worldwide common health problems. Vitamin D deficiency in adults has been associated with proximal muscle weakness, skeletal mineralization defect, and an increased risk of falling. Patients with vitamin D deficiency commonly complain of widespread pain in the body. The aim of this study was to examine the prevalence and risk factors of 25-hydroxyvitamin D deficiency in patients complaining of widespread musculoskeletal pain. Methods: In this cross-sectional study, 8457 patients with widespread musculoskeletal pain (7772 females, 685 males, aged 46.7 (range 20-100 years were included. Serum 25-hydroxyvitamin D was measured with ELISA method. Patients were classified into two groups: 1 Patients with vitamin D deficiency (20 ng/ml. Results: Prevalence of vitamin D deficiency was found to be 71.7%. A binary logistic regression model showed that low 25(OHVit D level was associated with gender, age and month in which 25(OH hypovitaminosis was determined. The risk of low 25(OH Vit D was found to be 2.15 times higher in female patients and 1.52 times higher on March and 1.55 times higher on April. Conclusion: This study indicates that Vitamin D deficiency should be taken into consideration in patients with widespread musculoskeletal pain, and some precautions such as sunbathe during summer should be recommended patients having risk of vitamin D deficiency. J Clin Exp Invest 2013; 4 (4: 48-491

  8. B-cell activating factor receptor deficiency is associated with an adult-onset antibody deficiency syndrome in humans

    OpenAIRE

    Warnatz, Klaus; Salzer, Ulrich; Rizzi, Marta; Fischer, Beate; Gutenberger, Sylvia; Böhm, Joachim; Kienzler, Anne-Kathrin; Pan-Hammarström, Qiang; Hammarström, Lennart; Rakhmanov, Mirzokhid; Schlesier, Michael; Grimbacher, Bodo; Peter, Hans-Hartmut; Eibel, Hermann

    2009-01-01

    B-cell survival depends on signals induced by B-cell activating factor (BAFF) binding to its receptor (BAFF-R). In mice, mutations in BAFF or BAFF-R cause B-cell lymphopenia and antibody deficiency. Analyzing BAFF-R expression and BAFF-binding to B cells in common variable immunodeficiency (CVID) patients, we identified two siblings carrying a homozygous deletion in the BAFF-R gene. Removing most of the BAFF-R transmembrane part, the deletion precludes BAFF-R expression. Without BAFF-R, B-cel...

  9. Characterization of MxFIT, an iron deficiency induced transcriptional factor in Malus xiaojinensis.

    Science.gov (United States)

    Yin, Lili; Wang, Yi; Yuan, Mudan; Zhang, Xinzhong; Xu, Xuefeng; Han, Zhenhai

    2014-02-01

    Iron deficiency often results in nutritional disorder in fruit trees. Transcription factors play an important role in the regulation of iron uptake. In this study, we isolated an iron deficiency response transcription factor gene, MxFIT, from an iron-efficient apple genotype of Malus xiaojinensis. MxFIT encoded a basic helix-loop-helix protein and contained a 966 bp open reading frame. MxFIT protein was targeted to the nucleus in onion epidermal cells and showed strong transcriptional activation in yeast cells. Spatiotemporal expression analysis revealed that MxFIT was up-regulated in roots under iron deficiency at both mRNA and protein levels, while almost no expression was detected in leaves irrespective of iron supply. Ectopic expression of MxFIT resulted in enhanced iron deficiency responses in Arabidopsis under iron deficiency and stronger resistance to iron deficiency. Thus, MxFIT might be involved in iron uptake and plays an important role in iron deficiency response.

  10. Vitamin D Deficiency : Universal Risk Factor for Multifactorial Diseases?

    NARCIS (Netherlands)

    de Borst, Martin H.; de Boer, Rudolf A.; Stolk, Ronald P.; Slaets, Joris P. J.; Wolffenbuttel, Bruce H. R.; Navis, Gerjan

    2011-01-01

    In the Western world, the majority of morbidity and mortality are caused by multifactorial diseases. Some risk factors are related to more than one type of disease. These so-called universal risk factors are highly relevant to the population, as reduction of universal risk factors may reduce the pre

  11. Vitamin D Deficiency: Universal Risk Factor for Multifactorial Diseases?

    NARCIS (Netherlands)

    de Borst, M.H.; de Boer, R.A.; Stolk, Ronald; Slaets, J.P J; Wolffenbuttel, B.H.R.; Navis, Ger Jan

    2011-01-01

    In the Western world, the majority of morbidity and mortality are caused by multifactorial diseases. Some risk factors are related to more than one type of disease. These so-called universal risk factors are highly relevant to the population, as reduction of universal risk factors may reduce the pre

  12. A novel mutation in a patient with congenital coagulation factordeficiency

    Institute of Scientific and Technical Information of China (English)

    FENG Ying; YE Xu; PANG Ying; DAI Jing; WANG Xue-feng; ZHOU Xu-hong

    2008-01-01

    @@ Human coagulation factor Ⅻ(FⅫ),also called Hageman factor,is a plasma plycoprotein that is functionally deficient in individuals with Hageman trait:which is an inhefited trait discovered by chance during preoperative blood coagulation screening tests.FⅫ is a single-chain 596-amino-acid zymogen of a serine protease with an approximate molecular weight of 80 000.

  13. Successful treatment of an acquired haemorrhagic diathesis due to factor X deficiency with chemotherapy

    NARCIS (Netherlands)

    de Jager, E; Bieger, R; Castel, A; Kluin, PM

    2001-01-01

    A 70-yr-old woman presented with a severe haemorrhagic diathesis due to an acquired factor X deficiency. A plasma infusion study showed that exogenous factor X was eliminated very effectively from the patient's circulation. A bone marrow biopsy was consistent with plasma cell dyscrasia. Neither an a

  14. Von Willebrand factor deficiency reduces liver fibrosis in mice

    NARCIS (Netherlands)

    Joshi, Nikita; Kopec, Anna K.; Ray, Jessica L.; Cline-Fedewa, Holly; Groeneveld, Dafna J.; Lisman, Ton; Luyendyk, James P.

    2017-01-01

    Liver diseases are associated with complex changes in the hemostatic system and elevated levels of the platelet adhesive protein Von Willebrand factor (VWF) are reported in patients with acute and chronic liver damage. Although elevated levels of VWF are associated with fibrosis in the general popul

  15. Prevalence and factors promoting the occurrence of vitamin D deficiency in the elderly

    Directory of Open Access Journals (Sweden)

    Magdalena Wyskida

    2017-03-01

    Full Text Available Vitamin D deficiency affects a large part of the population of elderly people, especially women, who live in moderate climate countries due to a reduced amount of vitamin D in the diet (small sea fish consumption and reduced content of 7-dehydrocholesterol, which causes decreased skin synthesis. The lowest seasonal concentration of 25(OHD3 is usually observed during winter and spring. Sun exposure influences 25(OHD3 concentration more strongly in men than in women.Sociodemographic factors that increase the risk of vitamin D deficiency in the elderly include poor environmental conditions, low economic status, lower educational level, drug exposure (smoking, reduced physical activity, overall poor health and obesity, which causes reduced skin exposure to sunlight.The use of medications or supplements that contain vitamin D and staying in a nursing home that employ such supplementation are factors that prevent deficiency.Significant prevalence of diseases of the gastrointestinal tract may contribute to cholecalciferol and ergocalciferol malabsorption or impair their liver transformation. In addition, the high incidence of chronic kidney disease in old age reduces processing hydroxylation of vitamin D and the formation of active metabolites.Vitamin D deficiency can not only cause bone mineralization disorders, but also increase incidence of cardiovascular diseases, cancers, type 2 diabetes and depression.The aim of this study was to summarize current knowledge about the risk factors of vitamin D deficiency development in the elderly population.

  16. Is vitamin B12 deficiency a risk factor for cardiovascular disease in vegetarians?

    Science.gov (United States)

    Pawlak, Roman

    2015-06-01

    The goal of this paper is to describe the role of vitamin B12 deficiency in cardiovascular disease development among vegetarians. Vegetarians have a high prevalence of vitamin B12 deficiency. Deficiency of this vitamin is associated with a variety of atherogenic processes that are mainly, but not exclusively, due to vitamin B12 deficiency-induced hyperhomocysteinemia. Each 5-μmol/L increase above 10 μmol/L of serum homocysteine is associated with a 20% increased risk of circulatory health problems. Mean homocysteine concentration >10 μmol/L among vegetarians was reported in 32 of 34 reports. Macrocytosis associated with vitamin B12 deficiency is also associated with fatal and non-fatal coronary disease, myocardial infarction, stroke, and other circulatory health problems. Compared with non-vegetarians, vegetarians have an improved profile of the traditional cardiovascular disease risk factors, including serum lipids, blood pressure, serum glucose concentration, and weight status. However, not all studies that assessed cardiovascular disease incidence among vegetarians reported a protective effect. Among studies that did show a lower prevalence of circulatory health problems, the effect was not as pronounced as expected, which may be a result of poor vitamin B12 status due to a vegetarian diet. Vitamin B12 deficiency may negate the cardiovascular disease prevention benefits of vegetarian diets. In order to further reduce the risk of cardiovascular disease, vegetarians should be advised to use vitamin B12 supplements. Copyright © 2015 American Journal of Preventive Medicine. Published by Elsevier Inc. All rights reserved.

  17. Einfluss der prophylaktischen Gabe von Faktor XIII auf die Wundheilung nach Neck Dissection bei Patienten mit chronischem Alkoholkonsum

    OpenAIRE

    Asmussen, Marie

    2016-01-01

    Wound healing disorders are a common complication in patients with Alcohol Use Disorder (AUD) after neck dissection. They can be differentiated into haematomas, seromas, wound dehiscences and wound infections. Important for wound healing are blood coagulation, an adequate immune response and tissue reformation. Factor XIII (FXIII) stabilises fibrin as well as collagene and supports fibroblasts and angiogenesis amongst others. Patients with AUD probably have a reduced level of FXlll. In thi...

  18. Iodine deficiency and associated factors among school children: a cross-sectional study in Ethiopia

    Directory of Open Access Journals (Sweden)

    Sintayehu Hailu

    2016-10-01

    Full Text Available Abstract Background Iodine deficiency remains a public health problem in the world. It is the leading cause of preventable mental retardation and brain damage worldwide. Though 12 million school age children are at risk of developing iodine deficiency, there is a scarcity of literature showing the magnitude of iodine deficiency in Ethiopia. Therefore, this study aimed to determine the prevalence and associated factors of iodine deficiency among school children in Robe District, southeast Ethiopia. Methods A school based cross–sectional study was conducted from February to June, 2015. A structured interviewer-administered questionnaire was used to collect data. A systematic random sampling technique was employed to select 422 children. A multivariate logistic regression analysis was carried out to identify factors associated with iodine deficiency. In the multivariate analysis, variables with a P-value of <0.05 were considered statistically significant. Results A total of 393 school children participated in the study. The median urinary iodine level was 78 μg/l. About 57 and 43.5 % of the children were found with low urinary iodine level and goiter, respectively. Only 29 % of the households utilized adequately iodized salt. The result of the multivariate analysis revealed that the odds of iodine deficiency were higher among female [AOR = 2.23; 95 % CI: 1.54, 3.55] and older (10–12 years [AOR = 2.21; 95 % CI: 1.44, 3.42] children. Conclusion In this community, the prevalence of goiter and low urine iodine level is high. Thus, iodine deficiency exists as severe public health problem. In addition, there is a low utilization of iodized salt in the setting. Therefore, it is crucial to intensify efforts in the implementation of iodized salt. Moreover, attention should be given to school children to address ID.

  19. Iodine deficiency and associated factors among school children: a cross-sectional study in Ethiopia.

    Science.gov (United States)

    Hailu, Sintayehu; Wubshet, Mamo; Woldie, Haile; Tariku, Amare

    2016-01-01

    Iodine deficiency remains a public health problem in the world. It is the leading cause of preventable mental retardation and brain damage worldwide. Though 12 million school age children are at risk of developing iodine deficiency, there is a scarcity of literature showing the magnitude of iodine deficiency in Ethiopia. Therefore, this study aimed to determine the prevalence and associated factors of iodine deficiency among school children in Robe District, southeast Ethiopia. A school based cross-sectional study was conducted from February to June, 2015. A structured interviewer-administered questionnaire was used to collect data. A systematic random sampling technique was employed to select 422 children. A multivariate logistic regression analysis was carried out to identify factors associated with iodine deficiency. In the multivariate analysis, variables with a P-value of iodine level was 78 μg/l. About 57 and 43.5 % of the children were found with low urinary iodine level and goiter, respectively. Only 29 % of the households utilized adequately iodized salt. The result of the multivariate analysis revealed that the odds of iodine deficiency were higher among female [AOR = 2.23; 95 % CI: 1.54, 3.55] and older (10-12 years) [AOR = 2.21; 95 % CI: 1.44, 3.42] children. In this community, the prevalence of goiter and low urine iodine level is high. Thus, iodine deficiency exists as severe public health problem. In addition, there is a low utilization of iodized salt in the setting. Therefore, it is crucial to intensify efforts in the implementation of iodized salt. Moreover, attention should be given to school children to address ID.

  20. Complement factor H deficiency and endocapillary glomerulonephritis due to paternal isodisomy and a novel factor H mutation

    DEFF Research Database (Denmark)

    Schejbel, L; Schmidt, I M; Kirchhoff, Eva Maria;

    2011-01-01

    Complement factor H (CFH) is a regulator of the alternative complement activation pathway. Mutations in the CFH gene are associated with atypical hemolytic uremic syndrome, membranoproliferative glomerulonephritis type II and C3 glomerulonephritis. Here, we report a 6-month-old CFH-deficient child...

  1. Genotype/phenotype correlations in complement factor h deficiency arising from uniparental isodisomy

    DEFF Research Database (Denmark)

    Wilson, Valerie; Darlay, Rebecca; Wong, William;

    2013-01-01

    We report a male infant who presented at 8 months of age with atypical hemolytic uremic syndrome (aHUS) responsive to plasma therapy. Investigation showed him to have complement factor H (CFH) deficiency associated with a homozygous CFH mutation (c.2880delT [p.Phe960fs]). Mutation screening of th...

  2. Coexistence of Factor VII Deficiency and Hereditary Spastic Paraplegia in Two Siblings

    Directory of Open Access Journals (Sweden)

    Hortensia De la Corte-Rodriguez

    2016-01-01

    Full Text Available We present the case of two patients aged 12 years and 7 years who were referred to our hospital for factor VII deficiency inherited in an autosomal recessive pattern, who had suffered from previous multiple joint haemarthroses. They presented with fine motor symptoms and difficulty in walking. During physical examination we observed neurological symptoms (general hypotonia, muscular hypotrophy, exaggerated tendon reflexes, pes cavus, and spastic gait. Given that the symptoms were not justified by the deficiency of coagulation factor VII and on suspicion of hereditary spastic paraplegia (HSP, tests were carried out. Findings from the tests confirmed the diagnosis of HSP (axonal degeneration of the central motor pathway and pyramidal tracts, further complicated by mixed neuropathy. This disease was also inherited in an autosomal recessive pattern with no direct genetic association with factor VII deficiency. Neurological symptoms had gone unnoticed due to a history of multiple joint haemarthrosis; musculoskeletal examination led to a satisfactory differential diagnosis. Haematological prophylaxis was commenced with rFVIIa at 30 mcg/kg, three days per week. A rehabilitation programme was prescribed so that the patient could remain independent for as long as possible, based on orthosis, physiotherapy, and occupational therapy. Response to treatment is currently satisfactory and no new bleeding has presented. As far as we are aware, the coexistence of these two diseases (factor VII deficiency and HSP has not been previously reported in the literature.

  3. The Complexity of the Puzzles of Final Fantasy XIII-2

    CERN Document Server

    Johnston, Nathaniel

    2012-01-01

    We analyze the computational complexity of solving the three "temporal rift" puzzles in the recent popular video game Final Fantasy XIII-2. We show that the Tile Trial puzzle is NP-hard and we provide an efficient algorithm for solving the Crystal Bonds puzzle. We also show that slight generalizations of the Crystal Bonds and Hands of Time puzzles are NP-hard.

  4. Importance of pharmacokinetic studies in the management of acquired factor X deficiency.

    Science.gov (United States)

    Lim, Ming Y; McCarthy, Timothy; Chen, Sheh-Li; Rollins-Raval, Marian A; Ma, Alice D

    2016-01-01

    Up to 14% of individuals with systemic AL amyloidosis develop acquired factor X deficiency, which occurs due to adsorption of factor X onto amyloid fibrils. Although baseline factor X levels are not predictive of bleeding risk in these patients, serious hemorrhagic complications can occur, particularly during invasive procedures. Optimal management strategies to attenuate bleeding risk in these patients are unknown. We describe our experience in the management of acquired factor X deficiency, secondary to systemic AL amyloidosis, in a case series of three patients who received prothrombin complex concentrates (PCCs) for treatment and prevention of bleeding events. We performed a retrospective review extracting information on baseline demographics, laboratory data, pharmacokinetic (PK) studies, and clinically documented bleeding events. Our case series demonstrates that individuals with acquired factor X deficiency secondary to amyloidosis have variable laboratory and clinical responses to PCCs. This is likely due to distinct amyloid loads and fibril sequences, leading to different binding avidities for factor X. Our data emphasize the importance of performing PK testing prior to any invasive procedures to determine the dose and frequency interval to achieve adequate factor X levels for hemostasis, given the variable response between individuals.

  5. Do PT and APTT sensitivities to factors' deficiencies calculated by the H47-A2 2008 CLSI guideline reflect the deficiencies found in plasmas from patients?

    Science.gov (United States)

    Martinuzzo, M; Barrera, L; Rodriguez, M; D'Adamo, M A; López, M S; Otaso, J C

    2015-12-01

    Prothrombin time (PT) and activated partial thromboplastin time (APTT) sensitivity for detecting isolated factor deficiencies varies with different reagents and coagulometers. The Clinical and Laboratory Standards Institute (CLSI) H47A2 guideline proposed a method to calculate these sensitivities, but some inconsistency has been reported. This study aimed to calculate factor sensitivities using CLSI guideline and to compare them with those obtained from single factor-deficient patients' data. Different mixtures of normal pooled and deficient plasmas were prepared (CLSI H47A2 guideline. PT with rabbit brain (RB) and human recombinant (HR) thromboplastins, APTT and factors' activities were measured in an ACL TOP coagulometer. Sensitivities (maximum factor concentration that produces PT or APTT values out of the reference range) were calculated from mixtures and from patients with single-factor deficiencies: 17 factor FV, 36 FVII, 19 FX, 39 FVIII, 15 FIX 15 FXI and 24 FXII. PT sensitivity with RB was as follows: FV 38 and 59, FVII 35 and 58, FX 56 and 64 IU/dL; PT sensitivity with HR was as follows: FV 39 and 45, FVII 51 and 50, FX 33 and 61 IU/dL; and APTT sensitivity was as follows: FV 39 and 45, FX 32 and 38, FVIII 47 and 60, FIX 35 and 44, FXI 33 and 43, FXII 37 and 46 IU/dL, respectively. Reagent-coagulometer combination has adequate sensitivities to factor deficiencies according to guideline recommendations (>30 IU/dL). These should not be considered as actual sensitivities because those obtained by analysing patients' plasmas with single-factor deficiencies were higher for most factors and could induce misinterpretation of the basic coagulation test results. © 2015 John Wiley & Sons Ltd.

  6. Prevalence of vitamin D deficiency and its associated factors in three regions of Saudi Arabia.

    Science.gov (United States)

    Kaddam, Ibrahim M; Al-Shaikh, Adnan M; Abaalkhail, Bahaa A; Asseri, Khalid S; Al-Saleh, Yousef M; Al-Qarni, Ali A; Al-Shuaibi, Ahmed M; Tamimi, Waleed G; Mukhtar, Abdelmoneim M

    2017-04-01

    To measure prevalence of vitamin D deficiency in Saudi Arabia, unveil the life style, nutritional habits and status, as well as identify the potential risk factors. Method: A school-based survey targeting Saudi school students and employees was conducted during the  period from 2013 to 2014 using multistage cluster random sample in Central, Western and Eastern regions. The prevalence of vitamin D deficiency and difference between various population subgroups were calculated. Logistic regression analysis was used to determine the predictors of potential risk factors. Results: Prevalence of vitamin D deficiency was 49.5% in students and 44% in employees. Life style was not adequate to protect against vitamin D depletion. Unhealthy nutritional habits were widespread, some manifested in childhood while others manifested later in life. Living in the Eastern region, females, 16-19 years of age, low economic class, obese and lack of omega 3 supplements were risk factors in students. Employees living in the Eastern region, females, middle-income class, carbonated soft drink consumers, and lack of multivitamin supplements were at higher risk. Conclusion: There is a need for a health awareness program using evidence-based recommendations. Screening for early detection and correction of the condition should be proposed to be part of the national health strategy. There is need for identifying the burden of vitamin D deficiency on other diseases to control and improve the prognosis of these conditions.

  7. Risk factors for goiter in a previously iodine-deficient region.

    Science.gov (United States)

    Völzke, H; Schwahn, C; Kohlmann, T; Kramer, A; Robinson, D M; John, U; Meng, W

    2005-10-01

    Little information exists from formerly iodine-deficient areas regarding gender-specific risk factors for goiter and their synergisms. The aim of the present study was to investigate such gender-specific risk factors and their interactions in a large population-based sample. The Study of Health in Pomerania (SHIP) comprised 4310 randomly selected participants, aged 20 - 79 years. SHIP was performed in a previously iodine-deficient region. Data from 3915 participants with no known thyroid disorders were analyzed. Goiter was determined by thyroid ultrasound. Sociodemographic characteristics, smoking and alcohol drinking habits, marital status, education level, urine thiocyanate concentrations, and specifically in women, parity and previous or current use of oral contraceptives and hormone replacement therapy, were considered as candidate risk factors for multivariable statistical tests. Only two variables, an advanced age and current smoking, were independently associated with an increased risk for goiter in both genders. Analyses further revealed specific risk factor profiles for goiter which were different among men, pre- and postmenopausal women. We conclude that besides previous iodine deficiency, other risk factors for goiter exist which differ between gender. Among the avoidable risk factors, current smoking was strongly associated with the risk of goiter in men and women. These findings should influence activities which are intended to prevent thyroid disease.

  8. Near fatal spontaneous intraperitoneal bleeding: A rare manifestation in a congenital factor X deficiency carrier

    Directory of Open Access Journals (Sweden)

    K V Vinod

    2015-01-01

    Full Text Available Congenital factor X (FX deficiency is a rare coagulation disorder of autosomal recessive inheritance, characterized by bleeding of variable severity. Bleeding severity generally correlates with the level of FX functional activity and severe bleeding usually occurs in moderate and severe deficiency, when FX coagulant activity is <5%. FX activity above 10% is infrequently associated with severe bleeding. Here we report the rare occurrence of life-threatening massive spontaneous intraperitoneal bleeding with hypovolemic shock, resulting from spontaneous rupture of an ovarian luteal cyst in a 25-year-old FX deficiency carrier woman, with a FX activity of 26%. She was managed successfully conservatively, with fresh frozen plasma and packed red blood cell transfusions and she showed gradual improvement. The case is being reported to discuss the diagnosis and management of this rare inherited coagulation disorder.

  9. Are there anamnestic risk factors for iron deficiency in pregnancy? Results from a feasibility study.

    Science.gov (United States)

    Kirschner, Wolf; Dudenhausen, Joachim W; Henrich, Wolfgang

    2016-04-01

    The conditions of iron deficiency are highly incident in pregnancy with elevated risks for preterm birth and low birth weight. In our recent study, we found 6% of participants having anemia, whereas between 39% and 47% showed iron deficiency without anemia. In many countries in prenatal care solely hemoglobin (Hb) measurement is applied. For the gynecologists till date there is no indication to determine other markers (e.g., serum-ferritin). As iron deficiency results from an imbalance between intake and loss of iron, our aim was to find out if the risk of iron deficiency conditions can be estimated by a diet history protocol as well as questionnaires to find about iron loss. We found that the risk of having iron deficiency in upper gestational week (>=21) increased by a factor of five. Thus, additional diagnostics should be done in this group by now. Using the questionnaire as a screening instrument, we further estimated the probability of disease in terms of a positive likelihood ratio (LR+). The positive LR for the group below 21th week of gestation is 1.9 thus, increasing the post-test probability to 52% from 36% as before. Further research based on higher sample sizes will show if the ratios can be increased further.

  10. Novel CFI mutation in a patient with leukocytoclastic vasculitis may redefine the clinical spectrum of Complement Factor I deficiency

    DEFF Research Database (Denmark)

    Bay, Jakob Thaning; Katzenstein, Terese Lea; Kofoed, Kristian

    2015-01-01

    presentation of Factor I deficiency varies and includes severe recurrent bacterial infections, glomerulonephritis and autoimmune diseases. The patient, a 28-years old woman with consanguineous parents, presented with recurrent leukocytoclastic vasculitis in the lower extremities with no associated systemic...... mutations vary among patients sole association with leukocytoclastic vasculitis redefines the clinical spectrum of complete Factor I deficiency....

  11. Risk factors associated with anemia and iron deficiency among Kuwaiti pregnant women.

    Science.gov (United States)

    Ahmed, Faruk; Al-Sumaie, Mona A

    2011-09-01

    A cross-sectional study was carried out to identify the risk factors of anemia and iron deficiency in Kuwaiti pregnant women. Pregnant women (n = 465) aged 18-47 years, of 4-39 weeks at gestation were recruited during antenatal visits from six health facilities in Kuwait. Socio-demographic, pregnancy-related and dietary information were collected. Hemoglobin, serum ferritin and serum C-reactive protein concentrations were determined. Logistic regression analysis revealed that iron deficiency and not taking iron-folate tablets or taking them occasionally were the two most important risk factors associated with anemia. Pregnant women with higher gestational age, short birth spacing ( ≤ 2 years), not taking iron-folate tablets or taking them occasionally, not consuming fruit juice, and consuming brown bread, tea and/or coffee were significant risk factors associated with iron deficiency. In conclusion, various factors including dietary habits appeared to be associated with poor iron status, which is the most important risk factor for anemia among Kuwaiti pregnant women.

  12. Fresh frozen plasma in the pediatric age group and in congenital coagulation factor deficiency.

    Science.gov (United States)

    Muntean, Wolfgang

    2002-10-31

    factor deficiency, replacement therapy is much more easily administered using a highly specific concentrate. When FFP is used to raise the level of the congenitally deficient factor, the huge volume needed to reach sufficiently high plasma levels can frequently be a major problem. For this reason, FFP as a replacement therapy in congenital factor deficiency is only indicated when no specific concentrate is available, as is the case in factor V deficiency and factor XI deficiency.

  13. Vitamin Deficiency Anemia

    Science.gov (United States)

    ... are unique to specific vitamin deficiencies. Folate-deficiency anemia risk factors include: Undergoing hemodialysis for kidney failure. ... the metabolism of folate. Vitamin B-12 deficiency anemia risk factors include: Lack of intrinsic factor. Most ...

  14. Lactase deficiency: a potential novel aetiological factor in chronic pruritus of unknown origin.

    Science.gov (United States)

    Grundmann, Sonja A; Stratmann, Ewelina; Brehler, Randolf; Luger, Thomas A; Ständer, Sonja

    2011-10-01

    Chronic pruritus, which is associated with a wide variety of underlying diseases, represents a challenge in diagnostics and treatment in dermatology and general medicine. The cause of pruritus remains unknown in up to 45% of patients. In this study, 718 patients with chronic pruritus were analysed concerning lactase deficiency, demographic data, aetiology, duration and intensity of pruritus. A total of 154 patients were tested positive for lactase deficiency and 38.3% showed a significant anti-pruritic response to a lactose-free diet (minimum 4 weeks). The best results were observed in patients with pruritus of mixed or unknown origin (n = 91; 64% response). Age, sex, localization or duration had no significant influence on the anti-pruritic effect of a lactose-free diet. Lactase deficiency might be an independent causal factor in the elicitation of chronic pruritus. Thus, screening for lactase deficiency represents a rational step in the diagnostic work-up of chronic pruritus. In case of a positive test result, a lactose-free diet offers a low-cost, efficient and specific therapy in patients with chronic pruritus.

  15. Decreased Lumbar Lordosis and Deficient Acetabular Coverage Are Risk Factors for Subchondral Insufficiency Fracture.

    Science.gov (United States)

    Jo, Woo Lam; Lee, Woo Suk; Chae, Dong Sik; Yang, Ick Hwan; Lee, Kyoung Min; Koo, Kyung Hoi

    2016-10-01

    Subchondral insufficiency fracture (SIF) of the femoral head occurs in the elderly and recipients of organ transplantation. Osteoporosis and deficient lateral coverage of the acetabulum are known risk factors for SIF. There has been no study about relation between spinopelvic alignment and anterior acetabular coverage with SIF. We therefore asked whether a decrease of lumbar lordosis and a deficiency in the anterior acetabular coverage are risk factors. We investigated 37 patients with SIF. There were 33 women and 4 men, and their mean age was 71.5 years (59-85 years). These 37 patients were matched with 37 controls for gender, age, height, weight, body mass index and bone mineral density. We compared the lumbar lordosis, pelvic incidence, pelvic tilt, sacral slope, acetabular index, acetabular roof angle, acetabular head index, anterior center-edge angle and lateral center-edge angle. Lumbar lordosis, pelvic tilt, sacral slope, lateral center edge angle, anterior center edge angle, acetabular index and acetabular head index were significantly different between SIF group and control group. Lumbar lordosis (OR = 1.11), lateral center edge angle (OR = 1.30) and anterior center edge angle (OR = 1.27) had significant associations in multivariate analysis. Decreased lumbar lordosis and deficient anterior coverage of the acetabulum are risk factors for SIF as well as decreased lateral coverage of the acetabulum.

  16. Informe sobre el XIII congreso internacional de Ahila

    Directory of Open Access Journals (Sweden)

    Fernando Mayorga García

    2010-08-01

    Full Text Available Informe sobre el XIII congreso internacional de Ahila , Azores,  Entre el 3 y el 6 de septiembre de 2002, en la Universidad de las Azores (Ponta Delgada, sesionó el XIII Congreso Internacional de la Asociación de Historiadores Latinoamericanistas Europeos (AHILA convocado bajo el lema "El Atlántico como eslabón de unión de pueblos: sociedades, políticas, economías y culturas". La conferencia inaugural tuvo lugar en el Complejo Científico de la Universidade dos Acores y estuvo a cargo del doctor Avelino de Freitas Meneses, quien se refirió al tema "Os Acores no centro do mundo: um propósito de reflexao histórica".

  17. The Double Didactic Astronomical Quadrant for the XIII International Astronomical Olympiad

    CERN Document Server

    Maris, Michele; Boehm, Conrad; Iafrate, Giulia; Ramella, Massimo

    2010-01-01

    Here we present the development of a simplified version of double astronomical quadrant, designed for educational aims and realized on the occasion of the observational round of the XIII International Astronomy Olympiad, held in Trieste (Italy) October 13-21, 2008. (Italia: In questo contributo illustriamo il progetto di una versione semplificata di doppio quadrante astronomico, progettato per fini didattici e realizzato in occasione dello svolgimento della gara osservativa delle XIII Olimpiadi Internazionali di Astronomia (XIII International Astronomy Olympiad, XIII IAO), Trieste (I), 13-21 ottobre 2008))

  18. Crónica de las XIII JAEM de Granada

    OpenAIRE

    Berenger, Luis

    2008-01-01

    Dentro de dos años, nos vemos en Gerona 2009... “He disfrutado muchísimo”, “Me he sentido muy a gusto en Granada”, “Días inolvidables”... Después de un curso muy largo y a pesar del fuerte calor; eran las 10 de la noche del 7 de Julio, tras la clausura de las XIII JAEM. Estas y otras cosas similares se podían oír.

  19. Role of hypoxia-inducible factor-1 in transcriptional activation of ceruloplasmin by iron deficiency

    Science.gov (United States)

    Mukhopadhyay, C. K.; Mazumder, B.; Fox, P. L.

    2000-01-01

    A role of the copper protein ceruloplasmin (Cp) in iron metabolism is suggested by its ferroxidase activity and by the tissue iron overload in hereditary Cp deficiency patients. In addition, plasma Cp increases markedly in several conditions of anemia, e.g. iron deficiency, hemorrhage, renal failure, sickle cell disease, pregnancy, and inflammation. However, little is known about the cellular and molecular mechanism(s) involved. We have reported that iron chelators increase Cp mRNA expression and protein synthesis in human hepatocarcinoma HepG2 cells. Furthermore, we have shown that the increase in Cp mRNA is due to increased rate of transcription. We here report the results of new studies designed to elucidate the molecular mechanism underlying transcriptional activation of Cp by iron deficiency. The 5'-flanking region of the Cp gene was cloned from a human genomic library. A 4774-base pair segment of the Cp promoter/enhancer driving a luciferase reporter was transfected into HepG2 or Hep3B cells. Iron deficiency or hypoxia increased luciferase activity by 5-10-fold compared with untreated cells. Examination of the sequence showed three pairs of consensus hypoxia-responsive elements (HREs). Deletion and mutation analysis showed that a single HRE was necessary and sufficient for gene activation. The involvement of hypoxia-inducible factor-1 (HIF-1) was shown by gel-shift and supershift experiments that showed HIF-1alpha and HIF-1beta binding to a radiolabeled oligonucleotide containing the Cp promoter HRE. Furthermore, iron deficiency (and hypoxia) did not activate Cp gene expression in Hepa c4 hepatoma cells deficient in HIF-1beta, as shown functionally by the inactivity of a transfected Cp promoter-luciferase construct and by the failure of HIF-1 to bind the Cp HRE in nuclear extracts from these cells. These results are consistent with in vivo findings that iron deficiency increases plasma Cp and provides a molecular mechanism that may help to understand these

  20. Genotype and phenotype correlation in intracranial hemorrhage in neonatal factor VII deficiency among Thai children

    Directory of Open Access Journals (Sweden)

    Traivaree C

    2017-06-01

    Full Text Available Chanchai Traivaree,1 Chalinee Monsereenusorn,1 Arunotai Meekaewkunchorn,2 Premsak Laoyookhong,3 Saranya Suwansingh,4 Boonchai Boonyawat5 1Division of Hematology/Oncology, Department of Pediatrics, Phramongkutklao Hospital and College of Medicine, 2Division of Hematology/Oncology, Department of Pediatrics, 3Division of Neonatology, Department of Pediatrics, Queen Sirikit National Institute of Child Health, Bangkok, 4Division of Hematology/Oncology, Department of Pediatrics, Chiangrai Prachanukroh Hospital, Chiang Rai, 5Division of Genetics, Department of Pediatrics, Phramongkutklao Hospital and College of Medicine, Bangkok, Thailand Abstract: Congenital factor VII (FVII deficiency is a rare inherited coagulopathy. The clinical manifestations and clinical findings vary widely, ranging from asymptomatic to life-threatening bleeding, including intracranial hemorrhage (ICH, with prolonged prothrombin time, normal partial thromboplastin time and normal platelet counts, which are confirmed by the low level of FVII assay. Treatment consists of fresh frozen plasma (FFP, prothrombin complex concentrates (PCCs, and recombinant activated FVII to treat bleeding and prophylactic therapy. Here, we report four patients with FVII levels <5% (severe type who presented ICH during the neonatal period. The IVS6+1G>T was the most common (50% mutation identified in our study, followed by the K376X nonsense mutation (37.5%. In our study, we found that genetic information affected the severity of congenital FVII deficiency with ICH. Keywords: mutation analysis, factor VII deficiency, Thai children

  1. The bHLH transcription factor POPEYE regulates response to iron deficiency in Arabidopsis roots.

    Science.gov (United States)

    Long, Terri A; Tsukagoshi, Hironaka; Busch, Wolfgang; Lahner, Brett; Salt, David E; Benfey, Philip N

    2010-07-01

    Global population increases and climate change underscore the need for better comprehension of how plants acquire and process nutrients such as iron. Using cell type-specific transcriptional profiling, we identified a pericycle-specific iron deficiency response and a bHLH transcription factor, POPEYE (PYE), that may play an important role in this response. Functional analysis of PYE suggests that it positively regulates growth and development under iron-deficient conditions. Chromatin immunoprecipitation-on-chip analysis and transcriptional profiling reveal that PYE helps maintain iron homeostasis by regulating the expression of known iron homeostasis genes and other genes involved in transcription, development, and stress response. PYE interacts with PYE homologs, including IAA-Leu Resistant3 (ILR3), another bHLH transcription factor that is involved in metal ion homeostasis. Moreover, ILR3 interacts with a third protein, BRUTUS (BTS), a putative E3 ligase protein, with metal ion binding and DNA binding domains, which negatively regulates the response to iron deficiency. PYE and BTS expression is also tightly coregulated. We propose that interactions among PYE, PYE homologs, and BTS are important for maintaining iron homeostasis under low iron conditions.

  2. Micronutrient Status among Pregnant Women in Zinder, Niger and Risk Factors Associated with Deficiency

    Directory of Open Access Journals (Sweden)

    K. Ryan Wessells

    2017-04-01

    Full Text Available Anemia and micronutrient (MN deficiencies in pregnant women are associated with adverse pregnancy outcomes. In Niger, 58.6% of pregnant women are anemic; however, MN statuses are unknown. The study objectives were to estimate the prevalence of MN deficiencies among pregnant women in Zinder, Niger and explore associated risk factors. Pregnant women living in randomly selected rural villages (n = 88 were included. Capillary and venous blood samples (n = 770 were analyzed for hemoglobin (Hb and plasma ferritin, soluble transferrin receptor (sTfR, zinc (pZn, retinol binding protein (RBP, folate and vitamin B12. C-reactive protein and alpha-1-acid glycoprotein were measured to adjust for inflammation. The prevalence of MN deficiencies in pregnant woman was high, indicative of a severe public health problem. Prevalence of iron deficiency was 20.7% and 35.7%, by ferritin (<15 µg/L and sTfR (>8.3 mg/L, respectively. In total, 40.7% of women had low pZn (<50 µg/dL, 79.7% had marginal RBP (<1.32 µmol/L, 44.3% of women had low folate (<10 nmol/L and 34.8% had low B12 concentrations (<148 pmol/L. Common risk factors associated with MN status included gravidity, mid-upper-arm circumference, geophagy, malaria, and result of the woman’s last pregnancy. Interventions to promote the strengthening of antenatal care, and access and adherence to nutrition and health interventions are critical among pregnant women in this population.

  3. Prevalence of Vitamin D Deficiency in Singapore: Its Implications to Cardiovascular Risk Factors.

    Directory of Open Access Journals (Sweden)

    Xinyan Bi

    Full Text Available Vitamin D deficiency is a global health challenge and has been linked to type 2 diabetes and other chronic diseases. However, the relationship between vitamin D status, body composition, and cardiovascular risks has not been well characterized in Asian populations. The objectives of this study were to examine the factors associated with the low vitamin D levels in a sunny tropical region and to assess the role of vitamin D status in cardiovascular risk factors.This was a cross-sectional study. One hundred and fourteen healthy participants (59 males and 55 females residing in Singapore took part in this study. Plasma 25OH-D3 concentration was measured by using LC-MS/MS. Body fat (% was measured by using three different techniques including bioelectrical impedance analysis (BIA, BOD POD, and dual-energy X-ray absorptiometry (DEXA. Basic anthropometric measurements, fasting blood glucose (FBG, fasting serum insulin (FSI, and lipid profiles were obtained using standard protocols.Approximately 42% of the participants were vitamin D deficient (< 20 ng/mL. Vitamin D status was inversely associated with body fat (%, homeostasis model assessment of insulin resistance (HOMA-IR, and total cholesterol/high density lipoprotein (TC/HDL ratio, while positively associated with lean body mass (LBM and hand grip strength (HGS.The high prevalence of vitamin D deficiency in a sunny tropical region reinforces the need to recognize that sunlight alone is not the precursor for optimal vitamin D status. This raises the need to investigate public health measures that will encourage exposure to sunlight without overexposure that is harmful to skin. More importantly, vitamin D deficiency is associated with increased cardiovascular risks, i.e. HOMA-IR, TC/HDL, and LDL/HDL. Future studies should attempt to elucidate the potential mechanisms.

  4. Complement factor I deficiency: a not so rare immune defect. Characterization of new mutations and the first large gene deletion

    Directory of Open Access Journals (Sweden)

    Alba-Domínguez María

    2012-06-01

    Full Text Available Abstract Background Complement Factor I (CFI is a serine protease with an important role in complement alternative pathway regulation. Complete factor I deficiency is strongly associated with severe infections. Approximately 30 families with this deficiency have been described worldwide. Patients and methods We have studied five new Spanish families suffering from CFI deficiency. From 19 screened people, 7 homozygous, 10 heterozygous and 2 healthy subjects were identified. Clinical, biochemical and genetic descriptions are included. Results Molecular studies demonstrated 4 novel mutations in the screened individuals; amongst them, we describe here the first great gene deletion reported in the CFI locus, which includes full exon 2 and part of the large intron 1. Conclusion CFI deficiency is possibly an underestimated defect and the eventual existence of this deficiency should be tested in those patients exhibiting low C3 and recurrent bacterial infections. We propose a simple diagnostic flowchart to help clinicians in the identification and correct diagnosis of such patients.

  5. Splenectomy and proximal lieno-renal shunt in a factor five deficient patient with extra-hepatic portal vein obstruction

    Directory of Open Access Journals (Sweden)

    Sahni Peush

    2006-05-01

    Full Text Available Abstract Background The clinico-surgical implication and successful management of a rare case of factor five (V deficiency with portal hypertension and hypersplenism due to idiopathic extra-hepatic portal venous obstruction is presented. Case presentation A 16-year old boy had gastro-esophageal variceal bleeding, splenomegaly and hypersplenism. During preoperative workup prolonged prothrombin time and activated partial thromboplastin time were detected, which on further evaluation turned out to be due to factor V deficiency. Proximal lieno-renal shunt and splenectomy were successfully performed with transfusion of fresh frozen plasma during and after the surgical procedure. At surgery there was no excessive bleeding. The perioperative course was uneventful and the patient is doing well on follow up. Conclusion Surgical portal decompressive procedures can be safely undertaken in clotting factor deficient patients with portal hypertension if meticulous surgical hemostasis is achieved at operation and the deficient factor is adequately replaced in the perioperative period.

  6. Myeloid differentiation factor 88 (MyD88-deficiency increases risk of diabetes in mice.

    Directory of Open Access Journals (Sweden)

    Toru Hosoi

    Full Text Available BACKGROUND: Multiple lines of evidence suggest innate immune response pathways to be involved in the development of obesity-associated diabetes although the molecular mechanism underling the disease is unknown. Recent observations suggest that saturated fatty acids can act as a ligand for toll-like receptor (TLR 4, which is thought to mediate obesity-associated insulin resistance. Myeloid differentiation factor 88 (MyD88 is an adapter protein for TLR/IL-1 receptor signaling, which is involved in the activation of inflammatory pathways. To evaluate molecular mechanisms linking obesity-associated diabetes down-stream of TLR4, we investigated physiological role of MyD88 in high-fat diet (HFD-induced obesity. METHODOLOGY/PRINCIPAL FINDINGS: In the present study, we found MyD88-deficient mice fed a HFD had increased circulating levels of insulin, leptin and cholesterol, as well as liver dysfunction (increased induction of ALT levels, increased activation of JNK and cleavage of PARP, which were linked to the onset of severe diabetes. On the other hand, TNF-alpha would not be involved in HFD-induced diabetes in MyD88-deficient mice, because TNF-alpha level was attenuated in MyD88-deficient mice fed with HFD. CONCLUSIONS/SIGNIFICANCE: The present finding of an unexpected role for MyD88 in preventing diabetes may provide a potential novel target/strategy for treating metabolic syndrome.

  7. Combined congenital dysfibrinogenemia and factor VII deficiency from mutations in the FGB and F7 genes.

    Science.gov (United States)

    Woo, Hye In; Park, In-Ae; Lee, Ki-O; Kim, Sun-Hee; Kim, Hee-Jin

    2012-07-01

    Dysfibrinogenemia and factor VII (FVII) deficiency are rare congenital coagulopathies. In this report, the authors describe a man with both defects confirmed by molecular genetic tests. The patient was a 51-year-old man referred for prolonged prothrombin time (PT) that had been accidentally detected on preoperative screening. He had no history of bleeding tendency even on occasions of surgery. Routine coagulation studies revealed prolonged PT (1.53 INR) and thrombin time (42.2 s), and decreased fibrinogen level (57 mg/dl) and FVII activity (44%). Direct sequencing analyses were performed on FGA, FGB, and FGG genes to confirm dysfibrinogenemia and on the F7 gene to confirm FVII deficiency. As a result, the patient was shown to be heterozygous for a point mutation in exon 8 of the FGB gene (c.1475A > G, p.*492Trpext*12; Fibrinogen Magdeburg II) and for a missense mutation in exon 6 of the F7 gene (c.466G  > A, p.Gly156Ser). To our knowledge, this is the first report on a case of combined dysfibrinogenemia and FVII deficiency confirmed by molecular genetic tests.

  8. Prevalence of factor VII deficiency and molecular characterization of the F7 gene in Brazilian patients.

    Science.gov (United States)

    Rodrigues, Dalva N; Siqueira, Lucia H; Galizoni, Andréa M; Arruda, Valder R; Annichino-Bizzacchi, Joyce M

    2003-04-01

    The prevalence of factor VII (FVII) deficiency in 267 Brazilian patients was estimated to be 4.1%, including one patient with significant bleeding, five with minor bleeding and five patients asymptomatic. Only one novel mutation 8926G F7 polymorphisms and FVII activity were found in these patients, as those with higher levels of FVII activity presented the genotype described in the literature as related to reduced FVII activity. As the R304Q mutation was the most frequent in these patients, and may be associated with an asymptomatic form of the disease, particularly in Blacks, we examined this mutation and FVII activity in 49 Blacks and 49 Caucasian blood donors with no clinical bleeding. None of the individuals showed the R304Q mutation, and FVII activity was normal in all of them, thus indicating that FVII deficiency is not common in normal individuals of these two ethnic groups in Brazil. This is the first study in South America to examine the prevalence and molecular basis of FVII deficiency, including the description of a novel mutation.

  9. Human conditions of insulin-like growth factor-I (IGF-I deficiency

    Directory of Open Access Journals (Sweden)

    Puche Juan E

    2012-11-01

    Full Text Available Abstract Insulin-like growth factor I (IGF-I is a polypeptide hormone produced mainly by the liver in response to the endocrine GH stimulus, but it is also secreted by multiple tissues for autocrine/paracrine purposes. IGF-I is partly responsible for systemic GH activities although it possesses a wide number of own properties (anabolic, antioxidant, anti-inflammatory and cytoprotective actions. IGF-I is a closely regulated hormone. Consequently, its logical therapeutical applications seems to be limited to restore physiological circulating levels in order to recover the clinical consequences of IGF-I deficiency, conditions where, despite continuous discrepancies, IGF-I treatment has never been related to oncogenesis. Currently the best characterized conditions of IGF-I deficiency are Laron Syndrome, in children; liver cirrhosis, in adults; aging including age-related-cardiovascular and neurological diseases; and more recently, intrauterine growth restriction. The aim of this review is to summarize the increasing list of roles of IGF-I, both in physiological and pathological conditions, underlying that its potential therapeutical options seem to be limited to those proven states of local or systemic IGF-I deficiency as a replacement treatment, rather than increasing its level upper the normal range.

  10. Hereditary juvenile cobalamin deficiency caused by mutations in the intrinsic factor gene.

    Science.gov (United States)

    Tanner, Stephan M; Li, Zhongyuan; Perko, James D; Oner, Cihan; Cetin, Mualla; Altay, Cigdem; Yurtsever, Zekiye; David, Karen L; Faivre, Laurence; Ismail, Essam A; Gräsbeck, Ralph; de la Chapelle, Albert

    2005-03-15

    Hereditary juvenile megaloblastic anemia due to vitamin B12 (cobalamin) deficiency is caused by intestinal malabsorption of cobalamin. In Imerslund-Grasbeck syndrome (IGS), cobalamin absorption is completely abolished and not corrected by the administration of intrinsic factor (IF); if untreated, the disease is fatal. Biallelic mutations either in the cubilin (CUBN) or amnionless (AMN) gene cause IGS. In a series of families clinically diagnosed with likely IGS, at least six displayed no evidence of mutations in CUBN or AMN. A genome-wide search for linkage followed by mutational analysis of candidate genes was performed in five of these families. A region in chromosome 11 showed evidence of linkage in four families. The gastric IF (GIF) gene located in this region harbored homozygous nonsense and missense mutations in these four families and in three additional families. The disease in these cases therefore should be classified as hereditary IF deficiency. Clinically, these patients resembled those with typical IGS; radiocobalamin absorption tests had been inconclusive regarding the nature of the defect. In the diagnosis of juvenile cobalamin deficiency, mutational analysis of the CUBN, AMN, and GIF genes provides a molecular characterization of the underlying defect and may be the diagnostic method of choice.

  11. The impact of thrombin generation and rotation thromboelastometry on assessment of severity of factor XI deficiency.

    Science.gov (United States)

    Livnat, Tami; Shenkman, Boris; Martinowitz, Uri; Zivelin, Ariella; Dardik, Rima; Tamarin, Ilia; Mansharov, Rachel; Budnik, Ivan; Salomon, Ophira

    2015-08-01

    The phenotype of bleeding in patients with severe FXI deficiency is unpredictable and unlike other bleeding disorders, it is not directly correlated with levels of FXI. In this study we analyzed whether the global coagulation assays can serve as a clinical tool in predicting bleeding tendency in patients with severe FXI deficiency undergoing surgery, taking into account the large inter-individual variability of FXI levels and genotypes. Thrombin generation (TG) was measured in 39 platelet-poor plasma with or without tissue factor (TF) and in the presence or absence of corn trypsin inhibitor (CTI). Rotation thromboelastometry (ROTEM) was performed with fresh whole blood of 26 patients applying NATEM and INTEM tests. TG induced by recalcification can distinguish between bleeding and non-bleeding patients with severe FXI deficiency particularly among those with FXI activity of 2-20IU/dl. The addition of TF or TF and CTI to the TG assay masked the ability to differentiate between XI activity, genotype as well as bleeding and non-bleeding patients. ROTEM assays failed to distinguish bleeding from non-bleeding patients but could do so between different FXI activity levels and genotypes. In conclusion, in the current study we found a sensitive tool to distinguish between bleeding and non-bleeding patients. In order to recommend TG as a predictive tool for treatment tailoring, a larger patient group is required.

  12. Fibroblast growth factor deficiencies impact anxiety-like behavior and the serotonergic system.

    Science.gov (United States)

    Brooks, Leah R; Enix, Courtney L; Rich, Samuel C; Magno, Jinno A; Lowry, Christopher A; Tsai, Pei-San

    2014-05-01

    Serotonergic neurons in the dorsal raphe nucleus (DR) are organized in anatomically distinct subregions that form connections with specific brain structures to modulate diverse behaviors, including anxiety-like behavior. It is unclear if the functional heterogeneity of these neurons is coupled to their developmental heterogeneity, and if abnormal development of specific DR serotonergic subregions can permanently impact anxiety circuits and behavior. The goal of this study was to examine if deficiencies in different components of fibroblast growth factor (Fgf) signaling could preferentially impact the development of specific populations of DR serotonergic neurons to alter anxiety-like behavior in adulthood. Wild-type and heterozygous male mice globally hypomorphic for Fgf8, Fgfr1, or both (Fgfr1/Fgf8) were tested in an anxiety-related behavioral battery. Both Fgf8- and Fgfr1/Fgf8-deficient mice display increased anxiety-like behavior as measured in the elevated plus-maze and the open-field tests. Immunohistochemical staining of a serotonergic marker, tryptophan hydroxylase (Tph), revealed reductions in specific populations of serotonergic neurons in the ventral, interfascicular, and ventrolateral/ventrolateral periaqueductal gray subregions of the DR in all Fgf-deficient mice, suggesting a neuroanatomical basis for increased anxiety-like behavior. Overall, this study suggests Fgf signaling selectively modulates the development of different serotonergic neuron subpopulations. Further, it suggests anxiety-like behavior may stem from developmental disruption of these neurons, and individuals with inactivating mutations in Fgf signaling genes may be predisposed to anxiety disorders.

  13. 40 CFR Appendix Xiii to Part 266 - Mercury Bearing Wastes That May Be Processed in Exempt Mercury Recovery Units

    Science.gov (United States)

    2010-07-01

    ... 40 Protection of Environment 26 2010-07-01 2010-07-01 false Mercury Bearing Wastes That May Be Processed in Exempt Mercury Recovery Units XIII Appendix XIII to Part 266 Protection of Environment... XIII to Part 266—Mercury Bearing Wastes That May Be Processed in Exempt Mercury Recovery Units These...

  14. Prophylaxis in congenital factor VII deficiency: indications, efficacy and safety. Results from the Seven Treatment Evaluation Registry (STER).

    Science.gov (United States)

    Napolitano, Mariasanta; Giansily-Blaizot, Muriel; Dolce, Alberto; Schved, Jean F; Auerswald, Guenter; Ingerslev, Jørgen; Bjerre, Jens; Altisent, Carmen; Charoenkwan, Pimlak; Michaels, Lisa; Chuansumrit, Ampaiwan; Di Minno, Giovanni; Caliskan, Umran; Mariani, Guglielmo

    2013-04-01

    Because of the very short half-life of factor VII, prophylaxis in factor VII deficiency is considered a difficult endeavor. The clinical efficacy and safety of prophylactic regimens, and indications for their use, were evaluated in factor VII-deficient patients in the Seven Treatment Evaluation Registry. Prophylaxis data (38 courses) were analyzed from 34 patients with severe factor VII deficiency (plasma-derived factor VII, and ten received fresh frozen plasma. Prophylactic schedules clustered into "frequent" courses (three times weekly, n=23) and "infrequent" courses (≤ 2 times weekly, n=15). Excluding courses for menorrhagia, "frequent" and "infrequent" courses produced 18/23 (78%) and 5/12 (41%) "excellent" outcomes, respectively; relative risk, 1.88; 95% confidence interval, 0.93-3.79; P=0.079. Long term prophylaxis lasted from 1 to >10 years. No thrombosis or new inhibitors occurred. In conclusion, a subset of patients with factor VII deficiency needed prophylaxis because of severe bleeding. Recombinant activated factor VII schedules based on "frequent" administrations (three times weekly) and a 90 μg/kg total weekly dose were effective. These data provide a rationale for long-term, safe prophylaxis in factor VII deficiency.

  15. Tierra y familia en la España meridional, siglos XIII-XIX.

    Directory of Open Access Journals (Sweden)

    Igor Goicovic Donoso

    2011-10-01

    Full Text Available Las siete contribuciones recopiladas por Francisco García Gon-zález en el texto Tierra y familia en la España meridional, siglos XIII-XIX, nos sitúan frente a la historia social a partir de dos inflexiones epistemológicas íntimamente relacionadas en la historia: tierra y familia. Efectivamente, la diada tierra-familia, durante el Antiguo Régimen europeo, se convirtió en un factor explicativo de buena parte de los procesos de articulación de la sociedad. Así, la relación de los sujetos con la propiedad y usufructo de la tierra, y las formas de articulación familiar desplegadas por los mismos, tienden a imbricarse recurrentemente en complejos procesos de reproducción social.

  16. Obesity and iron deficiency anemia as risk factors for asymptomatic bacteriuria.

    Science.gov (United States)

    Cuttitta, F; Torres, D; Vogiatzis, D; Buttà, C; Bellanca, M; Gueli, D; Lupo, U; Schimmenti, C; Virzì, G; Petrantoni, R; Balistreri, F; Paterna, S; Parrinello, G

    2014-03-01

    Few studies examined the risk factors of asymptomatic bacteriuria, showing contradictory results. Our study aimed to examine the association between different clinical and laboratory parameters and asymptomatic bacteriuria in internal medicine patients. 330 consecutive hospitalized subjects, asymptomatic for urinary tract infections (UTIs), underwent to microscopic examination of urine specimens. 100 subjects were positive for microscopic bacteriuria and were recruited into the study. At the quantitative urine culture 31 subjects of study population were positive while 69 subjects were negative for bacteriuria. The analysis of clinical characteristics showed that the two groups of subjects (positive and negative urine culture for bacteriuria) were significant different (pbacteriuria (Odds Ratios [OR]=3.79, p=0.0003; OR=2,65, p=0.0091; OR=2.63, p=0.0097; respectively). However, the multivariate analysis by logistic regression showed that only obesity and iron deficiency anemia, independently associated with positive urine culture for bacteriuria (OR=3.9695, p=0.0075; OR=3.1569, p=0.03420 respectively). This study shows that obesity and iron deficiency anemia are independent risk factors for asymptomatic bacteriuria. Copyright © 2014 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

  17. Los iura propria en Europa en el siglo XIII

    OpenAIRE

    Wolf, Armin

    1994-01-01

    La legislación es una conquista ya del siglo XIII, una época en la que se da el paso del derecho oral al derecho escrito. El autor elabora una sistematización conceptual y fijación del contenido de la variada termilogía normativa, a base de gráficos y ejemplos tomados de los diferentes países de Europa. Legislation is already an achievement in the 13th. century, epoch in which the step is taken from the oral law to the wnten one. The author elaborates a conceptual sistematiz...

  18. ZINC FINGER OF ARABIDOPSIS THALIANA12 (ZAT12) Interacts with FER-LIKE IRON DEFICIENCY-INDUCED TRANSCRIPTION FACTOR (FIT) Linking Iron Deficiency and Oxidative Stress Responses.

    Science.gov (United States)

    Le, Cham Thi Tuyet; Brumbarova, Tzvetina; Ivanov, Rumen; Stoof, Claudia; Weber, Eva; Mohrbacher, Julia; Fink-Straube, Claudia; Bauer, Petra

    2016-01-01

    Plants grown under iron (Fe)-deficient conditions induce a set of genes that enhance the efficiency of Fe uptake by the roots. In Arabidopsis (Arabidopsis thaliana), the central regulator of this response is the basic helix-loop-helix transcription factor FER-LIKE IRON DEFICIENCY-INDUCED TRANSCRIPTION FACTOR (FIT). FIT activity is regulated by protein-protein interactions, which also serve to integrate external signals that stimulate and possibly inhibit Fe uptake. In the search of signaling components regulating FIT function, we identified ZINC FINGER OF ARABIDOPSIS THALIANA12 (ZAT12), an abiotic stress-induced transcription factor. ZAT12 interacted with FIT, dependent on the presence of the ethylene-responsive element-binding factor-associated amphiphilic repression motif. ZAT12 protein was found expressed in the root early differentiation zone, where its abundance was modulated in a root layer-specific manner. In the absence of ZAT12, FIT expression was upregulated, suggesting a negative effect of ZAT12 on Fe uptake. Consistently, zat12 loss-of-function mutants had higher Fe content than the wild type at sufficient Fe. We found that under Fe deficiency, hydrogen peroxide (H2O2) levels were enhanced in a FIT-dependent manner. FIT protein, in turn, was stabilized by H2O2 but only in the presence of ZAT12, showing that H2O2 serves as a signal for Fe deficiency responses. We propose that oxidative stress-induced ZAT12 functions as a negative regulator of Fe acquisition. A model where H2O2 mediates the negative regulation of plant responses to prolonged stress might be applicable to a variety of stress conditions.

  19. Deficient leukemia inhibitory factor signaling in muscle precursor cells from patients with type 2 diabetes

    DEFF Research Database (Denmark)

    Broholm, Christa; Brandt, Claus; Schultz, Ninna S

    2012-01-01

    The cytokine leukemia-inhibitory factor (LIF) is expressed by skeletal muscle and induces proliferation of muscle precursor cells, an important feature of skeletal muscle maintenance and repair. We hypothesized that muscle precursor cells from patients with type 2 diabetes had a deficient response......-stimulated cell proliferation and a decreased LIF-stimulated induction of the proliferation-promoting factors cyclin D1, JunB, and c-myc. SOCS3 protein was upregulated in diabetic myoblasts, and knockdown of SOCS3 rescued LIF-induced gene expression in diabetic myoblasts, whereas neither STAT1 or STAT3 signaling...... nor proliferation rate was affected. In conclusion, although LIF and LIFR proteins were increased in muscle tissue and myoblasts from diabetic patients, LIF signaling and LIF-stimulated cell proliferation were impaired in diabetic myoblasts, suggesting a novel mechanism by which muscle function...

  20. [Recurrence of Waldenström macroglobulinemia accompanied by factor X deficiency].

    Science.gov (United States)

    Ohara, Shin; Hagihara, Masao; Hua, Jian; Inoue, Morihiro; Uchida, Tomoyuki; Yoshinaga, Tsuneaki; Yazaki, Masahide; Sekijima, Yoshiki; Kametani, Fuyuki

    2016-03-01

    A medical check-up revealed severe anemia in an 85-year-old man who had been diagnosed with Waldenström macroglobulinemia 11 years previously. On the other hand, prolonged PT and aPTT were demonstrated on admission, and were attributed to a significant decrease in factor X activity. These abnormalities were all considered to be have been caused by an exacerbation of the underlying disease and, thus, chemotherapy with the RCD regimen (rituximab, cyclophosphamide, dexamethasone) was started. No significant improvement was obtained and the patient died suddenly on day 154. AL amyloidosis was diagnosed by histopathological examinations and also confirmed by a sequence analysis of amyloid protein. This case with Waldenström macroglobulinemia complicated by AL amyloidosis and recurrent factor X deficiency is quite rare.

  1. Construction of a mouse model of factor VIII deficiency by gene targeting

    Energy Technology Data Exchange (ETDEWEB)

    Bi, L.; Lawler, A.; Gearhart, J. [Univ. of Pennsylvania School of Medicine, Philadelphia, PA (United States)] [and others

    1994-09-01

    To develop a small animal model of hemophilia A for gene therapy experiments, we set out to construct a mouse model for factor VIII deficiency by gene targeting. First, we screened a mouse liver cDNA library using a human FVIII cDNA probe. We cloned a 2.6 Kb partial mouse factor VIII cDNA which extends from 800 base pairs of the 3{prime} end of exon 14 to the 5{prime} end of exon 26. A mouse genomic library made from strain 129 was then screened to obtain genomic fragments covering the exons desired for homologous recombination. Two genomic clones were obtained, and one covering exon 15 through 22 was used for gene targeting. To make gene targeting constructs, a 5.8 Kb genomic DNA fragment covering exons 15 to 19 of the mouse FVIII gene was subcloned, and the neo expression cassette was inserted into exons 16 and 17 separately by different strategies. These two constructs were named MFVIIIC-16 and MFVIIIC-17. The constructs were linearized and transfected into strain 129 mouse ES cells by electroporation. Factor VIII gene-knockout ES cell lines were selected by G-418 and screened by genomic Southern blots. Eight exon 16 targeted cell lines and five exon 17 targeted cell lines were obtained. Three cell lines from each construct were injected into blastocysts and surgically transferred into foster mothers. Multiple chimeric mice with 70-90% hair color derived from the ES-cell genotype were seen with both constructs. Germ line transmission of the ES-cell genotype has been obtained for the MFVIIIC-16 construct, and multiple hemophilia A carrier females have been identified. Factor VIII-deficient males will be conceived soon.

  2. Inflammation and functional iron deficiency regulate fibroblast growth factor 23 production.

    Science.gov (United States)

    David, Valentin; Martin, Aline; Isakova, Tamara; Spaulding, Christina; Qi, Lixin; Ramirez, Veronica; Zumbrennen-Bullough, Kimberly B; Sun, Chia Chi; Lin, Herbert Y; Babitt, Jodie L; Wolf, Myles

    2016-01-01

    Circulating levels of fibroblast growth factor 23 (FGF23) are elevated in patients with chronic kidney disease (CKD), but the mechanisms are poorly understood. Here we tested whether inflammation and iron deficiency regulate FGF23. In wild-type mice, acute inflammation induced by single injections of heat-killed Brucella abortus or interleukin-1β (IL-1β) decreased serum iron within 6 h, and was accompanied by significant increases in osseous Fgf23 mRNA expression and serum levels of C-terminal FGF23, but no changes in intact FGF23. Chronic inflammation induced by repeated bacteria or IL-1β injections decreased serum iron, increased osseous Fgf23 mRNA, and serum C-terminal FGF23, but modestly increased biologically active, intact FGF23 serum levels. Chronic iron deficiency mimicked chronic inflammation. Increased osseous FGF23 cleavage rather than a prolonged half-life of C-terminal FGF23 fragments accounted for the elevated C-terminal FGF23 but near-normal intact FGF23 levels in inflammation. IL-1β injection increased Fgf23 mRNA and C-terminal FGF23 levels similarly in wildtype and Col4a3(ko) mice with CKD but markedly increased intact FGF23 levels only in the CKD mice. Inflammation increased Fgf23 transcription by activating Hif1α signaling. Thus, inflammation and iron deficiency stimulate FGF23 production. Simultaneous upregulation of FGF23 cleavage in osteocytes maintains near-normal levels of biologically active, intact circulating FGF23, whereas downregulated or impaired FGF23 cleavage may contribute to elevated intact serum FGF23 in CKD.

  3. Systemic and intestinal levels of factor XIII-A

    DEFF Research Database (Denmark)

    Soendergaard, Christoffer; Kvist, Peter Helding; Seidelin, Jakob Benedict

    2016-01-01

    : Increased infiltration of CD68 positive macrophages in the inflamed mucosa coincided with increased extracellular deposited FXIII-A and decreased expression and intracellular protein levels of FXIII-A. A decreased proportion of FXIII-A/CD68/CD163 triple-positive macrophages was observed in inflamed mucosa......, indicating a reduction of the M2 phenotype with consequent loss of FXIII-A. No induction of iNOS positive macrophages was observed. Stimulation of naïve monocytes with physiological concentrations of pro-inflammatory mediators negatively affected the expression of FXIII-A. Measurements in plasma confirmed...

  4. Factors associated with iron deficiency in pregnant women seen at a public prenatal care service

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    Rosângela Maria Souza de Camargo

    2013-08-01

    Full Text Available OBJECTIVE: This study aimed to determine the frequency of iron deficiency and its association with socioeconomic, obstetric, and nutritional factors in pregnant women. METHODS: This cross-sectional study included women on the second trimester of pregnancy seen at a public prenatal care facility of Cuiabá, Mato Grosso, Brazil from May 2008 to May 2009. Socioeconomic, nutritional, and obstetric data were compared with markers of iron stores. RESULTS: During the study period, 146 pregnant women met the inclusion criteria. The frequency of anemia characterized by abnormal hemoglobin level, hematocrit, and mean corpuscular volume varied from 3% to 5%. However, 11% of the women had high transferrin levels and 39% had low ferritin levels. Before pregnancy, 21% of the women were underweight and 29% were overweight or obese. During pregnancy, the percentage of overweight or obese women rose to 40%. History of miscarriages, higher gestational age, and excess weight before pregnancy were associated with markers of iron stores at abnormal levels. Consumption of specific food groups was not associated with abnormal marker levels. CONCLUSION: Serum ferritin was the most sensitive indicator of iron deficiency. Excess weight and anemia were concomitant.

  5. The effects of maternal iron deficiency on infant fibroblast growth factor-23 and mineral metabolism.

    Science.gov (United States)

    Braithwaite, V S; Prentice, A; Darboe, M K; Prentice, A M; Moore, S E

    2016-02-01

    Fibroblast growth factor-23 (FGF23), a phosphate(Phos)-regulating hormone, is abnormally elevated in hypophosphataemic syndromes and an elevated FGF23 is a predictor of mortality in kidney disease. Recent findings suggest iron deficiency as a potential mediator of FGF23 expression and murine studies have shown in utero effects of maternal iron deficiency on offspring FGF23 and phosphate metabolism. Our aim was to investigate the impact of maternal iron status on infant FGF23 and mineral metabolites over the first 2years of life. Infants born to mothers with normal (NIn=25,) and low (LIn=25) iron status during pregnancy, from a mother-infant trial (ISRCTN49285450) in rural Gambia, West Africa, had blood and plasma samples analysed at 12, 24, 52, 78 and 104weeks (wk) of age. Circulating intact-FGF23 (I-FGF23), Phos, total alkaline phosphatase (TALP) and haemoglobin (Hb) decreased and estimated glomerular filtration rate increased over time [all P≤0.0001)]. C-terminal-FGF23 (C-FGF23) and TALP were significantly higher in LI compared with NI, from 52wk for C-FGF23 [Beta coefficient (SE) 18.1 (0.04) %, P=0.04] and from 24wk for TALP [44.7 (29.6) U/L, P=0.04]. Infant Hb was the strongest negative predictor of C-FGF23 concentration [-21% (4%) RU/mL, P≤0.0001], Phos was the strongest positive predictor of I-FGF23 [32.0(3.9) pg/mL, P≤0.0001] and I-FGF23 did not predict C-FGF23 over time [-0.5% (0.5%), P=0.3]. In conclusion, this study suggests that poor maternal iron status is associated with a higher infant C-FGF23 and TALP but similar I-FGF23 concentrations in infants and young children. These findings further highlight the likely public health importance of preventing iron deficiency during pregnancy. Whether or not children who are born to iron deficient mothers have persistently high concentrations of these metabolites and are more likely to be at risk of impaired bone development and pre-disposed to rickets requires further research.

  6. Vitamin D Deficiency among Female Nurses of Children’s Medical Center Hospital and Its Related Factors

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    Hamid Rajebi

    2016-03-01

    Full Text Available Vitamin D deficiency is one of the most preventable challenges worldwide. The aim of this study was to determine the prevalence of vitamin D deficiency among female nurses working at Children’s Medical Center Hospital in Tehran, Iran, due to the risk factor of being a notably long period indoors and the fact that their health status may have consequences on the process of patients’ treatment. A total of 114 female nurses who were at least 20 years old entered the study voluntarily, and a questionnaire was applied to collect information on lifestyle and other factors associated with vitamin D deficiency. A sample of blood was taken to measure 25-hydroxyvitamin D (25-OHD and cut off value to indicate deficiency was considered below 10ng/ml, and the amounts of 10-29ng/ml were declared insufficient. The mean of 25-OHD was 11.7±9.3ng/ml. A total of 79 subjects (69.3% had a deficient level of vitamin D, 28 subjects (24.6% had an insufficient level and only 7 subjects (6.1% had sufficient level of vitamin D. The deficiency was more noticeable in the age group of 26-35 years old. Prevalence of vitamin D deficiency had a significant correlation with younger subjects (P<0.001. There was no significant association among other factors such as body mass index (BMI, health status complications, regular exercise, and duration of sun exposure. High prevalence of vitamin D deficiency in the study population leads to emphasise the need to screen health care workers for vitamin D levels.

  7. The evaluation of iron deficiency and anemia in male blood donors with other related factors

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    Yousefinejad Vahid

    2010-01-01

    Full Text Available Aims and Background: Iron deficiency is one of the most common nutritional disorders worldwide and blood donation may cause iron depletion. Limited studies with large sample size have been done on male donors. The aim of this study is to determine the prevalence of iron deficiency and iron deficiency anemia among male donors in the Kurdistan Organization of Blood Transfusion in Iran. Materials and Methods: This was a cross-sectional study. Sample size was 1184 blood donors selected by systematic random sampling. Hemoglobin, serum iron, serum ferritin, total iron banding capacity (TIBC and transferin saturation were measured in donors. Iron depletion, lack of iron stores, iron deficiency, iron deficiency anemia and anemia were evaluated among them. Data was analyzed with SPSS software and X΂, one-way ANOVA, and LSD test. Results: Iron deficiency, anemia, iron deficiency anemia, iron depletion and lack of iron resources were seen in 2.3, 4.08, 2.14, 22.76 and 4.66 percent respectively. There was a significant relationship of iron deficiency and iron deficiency anemia with instances of donation and interval from last donation (P < 0.05. A significant relationship was seen between iron deficiency and iron deficiency anemia among blood donors with more than ten times blood donation (P < 0.05. Conclusions: This study showed regular male donors require especial attention. Therefore, serum ferritin is recommended as a more adequate index to use for iron deficiency screening and planning purposes for iron supplementation among them.

  8. Use of pharmacokinetic modelling to individualize FFP dosing in factor V deficiency.

    Science.gov (United States)

    Shakhnovich, V; Daniel, J; Wicklund, B; Kearns, G; Neville, K

    2013-03-01

    Therapy with fresh frozen plasma (FFP) confers serious risks, such as contraction of blood-borne viruses, allergic reaction, volume overload and development of alloantibodies. The aim of this study was to apply principles of pharmacokinetic (PK) modelling to individual factor content of FFP to optimize individualized dosing, while minimizing potential risks of therapy. We used PK modelling to successfully target individual factor replacement in an 8-month-old patient receiving FFP for treatment of a severe congenital factor V (FV) deficiency. The model fit for the FV activity vs. time data was excellent (r = 0.98) and the model accurately predicted FV activity during the intraoperative and postoperative period. Accurate PK modelling of individual factor activity in FFP has the potential to provide better targeted therapy, enabling clinicians to more precisely dose patients requiring coagulation products, while avoiding wasteful and expensive product overtreatment, minimizing potentially life-threatening complications due to undertreatment and limiting harmful product-associated risks.

  9. Social capital of Iranian patients living with acquired immune deficiency syndrome and associated factors.

    Science.gov (United States)

    Ansari, S K; Nedjat, S; Jabbari, H; Saiepour, N; Heris, M J

    2015-12-13

    This study investigated the social capital of Iranian patients living with acquired immune deficiency syndrome (AIDS) and the associated factors. In a cross-sectional study the Integrated Social Capital Questionnaire was filled by a sequential sample of 300 patients visiting a referral counselling centre in Tehran. The patients' social capital scores were around 50% in the trust, social cohesion, collective action and cooperation and political empowerment domains. The groups and networks membership domain scored the lowest (27.1%). In regression analysis, employment status was significantly associated with groups and networks membership; age, marital status and financial status were associated with collective action and cooperation; period of disease awareness and marital status affected social cohesion and inclusion; and having risky behaviour affected empowerment and political action. Efforts are needed to enhance the social capital of those patients living with AIDS who are younger, unemployed, divorced/widowed, with risky behaviours and shorter disease awareness.

  10. Identifying training deficiencies in military pilots by applying the human factors analysis and classification system.

    Science.gov (United States)

    Li, Wen-Chin; Harris, Don

    2013-01-01

    Without accurate analysis, it is difficult to identify training needs and develop the content of training programs required for preventing aviation accidents. The human factors analysis and classification system (HFACS) is based on Reason's system-wide model of human error. In this study, 523 accidents from the Republic of China Air Force were analyzed in which 1762 human errors were categorized. The results of the analysis showed that errors of judgment and poor decision-making were commonly reported amongst pilots. As a result, it was concluded that there was a need for military pilots to be trained specifically in making decisions in tactical environments. However, application of HFACS also allowed the identification of systemic training deficiencies within the organization further contributing to the accidents observed.

  11. Prevalence and risk factors of iron deficiency in healthy young children in the southwestern Netherlands.

    Science.gov (United States)

    Uijterschout, Lieke; Vloemans, Janneke; Vos, Rimke; Teunisse, Peter-Paul; Hudig, Cisca; Bubbers, Sally; Verbruggen, Sascha; Veldhorst, Margriet; de Leeuw, Tom; van Goudoever, Johannes B; Brus, Frank

    2014-02-01

    Iron deficiency (ID) and iron deficiency anemia (IDA), during the first years of life, are associated with delayed motor and neurological development. Many studies evaluated iron status without an assessment of an acute-phase protein to identify infection. Because most indicators of iron status are influenced by infection, these data may underestimate the ID prevalence. A food consumption survey in the Netherlands showed that the mean iron intake of children ages 2 to 3 years was below the advised adequate intake of 7 mg/day. The aim of the study was to investigate iron status in a well-defined, healthy population of young children in the southwestern region of the Netherlands and to identify risk factors for ID. We conducted a multicenter, observational study in healthy children ages 0.5 to 3 years. We defined ID as ferritin Children with elevated C-reactive protein levels (>5 mg/L) or underlying causes for anemia were excluded. Parents filled in a questionnaire to identify risk factors for ID. We included 400 children in the study. ID and IDA were detected in 18.8% and 8.5% of the children, respectively. The present use of formula and the visit of preschool/day care were associated with a lower prevalence of ID, and a high intake of cow's milk was associated with a higher prevalence of ID, after adjustment for age. ID is present in 18.8% of healthy children ages 0.5 to 3 years and living in the southwestern region of the Netherlands. The present visit of preschool/day care and the use of formula are associated with a reduced risk of ID, whereas a high intake of cow's milk is associated with an increased risk of ID.

  12. [Iodine nutritional status and risk factors for iodine deficiency in infants and children of the french North department].

    Science.gov (United States)

    Pouessel, G; Bouarfa, K; Soudan, B; Sauvage, J; Gottrand, F; Turck, D

    2003-02-01

    Iodine deficiency is responsible for a higher mortality and morbidity in neonates and infants. It has not yet disappeared in European countries, especially in Southern and Eastern Europe. The present study aimed at evaluating the status of iodine nutrition of infants living in the North department (France) and at studying risk factors for iodine deficiency. The study was conducted in primary health care centres in 160 healthy infants aged ten days to six years (mean +/- SD: 17.7 +/- 2.5 months). Data included: familial thyroid disease history, type of feeding at inclusion, timing of introduction of complementary foods, nutritional status (weight, height, head and arm circumference), as well as maternal education level and family socio-economical status. Iodine status was assessed by urinary iodine excretion. Urinary iodine concentration ranged from 4 to 1042 microg/l (median +/- SD: 195,5 +/- 21,6 microg/l). Thirty-eight (24%) of 160 children were iodine deficient (urinary iodine iodine deficiency (50-99 microg/l: 17%), moderate iodine deficiency (20-49 microg/l: 5%), severe iodine deficiency (iodine status and age, sex, geographic origin of the children, as well as social and occupational group of the parents. Breast-feeding did not prevent from iodine deficiency. Iodine status did not differ between the cow's milk fed group and the group that was not fed cow's milk. Formula feeding was associated with iodine deficiency (p = 0,02). Prevalence of severe iodine deficiency was very low in this population. However, iodine status was not optimal.

  13. Factor XI Deficiency Alters the Cytokine Response and Activation of Contact Proteases during Polymicrobial Sepsis in Mice.

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    Charles E Bane

    Full Text Available Sepsis, a systemic inflammatory response to infection, is often accompanied by abnormalities of blood coagulation. Prior work with a mouse model of sepsis induced by cecal ligation and puncture (CLP suggested that the protease factor XIa contributed to disseminated intravascular coagulation (DIC and to the cytokine response during sepsis. We investigated the importance of factor XI to cytokine and coagulation responses during the first 24 hours after CLP. Compared to wild type littermates, factor XI-deficient (FXI-/- mice had a survival advantage after CLP, with smaller increases in plasma levels of TNF-α and IL-10 and delayed IL-1β and IL-6 responses. Plasma levels of serum amyloid P, an acute phase protein, were increased in wild type mice 24 hours post-CLP, but not in FXI-/- mice, supporting the impression of a reduced inflammatory response in the absence of factor XI. Surprisingly, there was little evidence of DIC in mice of either genotype. Plasma levels of the contact factors factor XII and prekallikrein were reduced in WT mice after CLP, consistent with induction of contact activation. However, factor XII and PK levels were not reduced in FXI-/- animals, indicating factor XI deficiency blunted contact activation. Intravenous infusion of polyphosphate into WT mice also induced changes in factor XII, but had much less effect in FXI deficient mice. In vitro analysis revealed that factor XIa activates factor XII, and that this reaction is enhanced by polyanions such polyphosphate and nucleic acids. These data suggest that factor XI deficiency confers a survival advantage in the CLP sepsis model by altering the cytokine response to infection and blunting activation of the contact (kallikrein-kinin system. The findings support the hypothesis that factor XI functions as a bidirectional interface between contact activation and thrombin generation, allowing the two processes to influence each other.

  14. [Prevalence of vitamin D deficiency and associated factors in women and newborns in the immediate postpartum period].

    Science.gov (United States)

    do Prado, Mara Rúbia Maciel Cardoso; Oliveira, Fabiana de Cássia Carvalho; Assis, Karine Franklin; Ribeiro, Sarah Aparecida Vieira; do Prado Junior, Pedro Paulo; Sant'Ana, Luciana Ferreira da Rocha; Priore, Silvia Eloiza; Franceschini, Sylvia do Carmo Castro

    2015-01-01

    To assess the prevalence of vitamin D deficiency and its associated factors in women and their newborns in the postpartum period. This cross-sectional study evaluated vitamin D deficiency/insufficiency in 226 women and their newborns in Viçosa (Minas Gerais, BR) between December 2011 and November 2012. Cord blood and venous maternal blood were collected to evaluate the following biochemical parameters: vitamin D, alkaline phosphatase, calcium, phosphorus and parathyroid hormone. Poisson regression analysis, with a confidence interval of 95% was applied to assess vitamin D deficiency and its associated factors. Multiple linear regression analysis was performed to identify factors associated with 25(OH)D deficiency in the newborns and women from the study. The criteria for variable inclusion in the multiple linear regression model was the association with the dependent variable in the simple linear regression analysis, considering p<0.20. Significance level was α<5%. From 226 women included, 200 (88.5%) were 20 to 44 years old; the median age was 28 years. Deficient/insufficient levels of vitamin D were found in 192 (85%) women and in 182 (80.5%) neonates. The maternal 25(OH)D and alkaline phosphatase levels were independently associated with vitamin D deficiency in infants. This study identified a high prevalence of vitamin D deficiency and insufficiency in women and newborns and the association between maternal nutritional status of vitamin D and their infants' vitamin D status. Copyright © 2015 Sociedade de Pediatria de São Paulo. Publicado por Elsevier Editora Ltda. All rights reserved.

  15. Factor XI-deficient mice display reduced inflammation, coagulopathy, and bacterial growth during listeriosis.

    Science.gov (United States)

    Luo, Deyan; Szaba, Frank M; Kummer, Lawrence W; Johnson, Lawrence L; Tucker, Erik I; Gruber, Andras; Gailani, David; Smiley, Stephen T

    2012-01-01

    In mice infected sublethally with Listeria monocytogenes, fibrin is deposited at low levels within hepatic tissue, where it functions protectively by limiting bacterial growth and suppressing hemorrhagic pathology. Here we demonstrate that mice infected with lethal doses of L. monocytogenes produce higher levels of fibrin and display evidence of systemic coagulopathy (i.e., thrombocytopenia, fibrinogen depletion, and elevated levels of thrombin-antithrombin complexes). When the hepatic bacterial burden exceeds 1×10(6) CFU, levels of hepatic fibrin correlate with the bacterial burden, which also correlates with levels of hepatic mRNA encoding the hemostatic enzyme factor XI (FXI). Gene-targeted FXI-deficient mice show significantly improved survival upon challenge with high doses of L. monocytogenes and also display reduced levels of hepatic fibrin, decreased evidence of coagulopathy, and diminished cytokine production (interleukin-6 [IL-6] and IL-10). While fibrin limits the bacterial burden during sublethal listeriosis in wild-type mice, FXI-deficient mice display a significantly improved capacity to restrain the bacterial burden during lethal listeriosis despite their reduced fibrin levels. They also show less evidence of hepatic necrosis. In conjunction with suboptimal antibiotic therapy, FXI-specific monoclonal antibody 14E11 improves survival when administered therapeutically to wild-type mice challenged with high doses of L. monocytogenes. Together, these findings demonstrate the utility of murine listeriosis as a model for dissecting qualitative differences between protective and pathological host responses and reveal novel roles for FXI in exacerbating inflammation and pathogen burden during a lethal bacterial infection.

  16. Effects of growth hormone and insulin-like growth factor 1 deficiency on ageing and longevity.

    Science.gov (United States)

    Laron, Zvi

    2002-01-01

    Present knowledge on the effects of growth hormone (GH)/insulin-like growth hormone (IGF)1 deficiency on ageing and lifespan are reviewed. Evidence is presented that isolated GH deficiency (IGHD), multiple pituitary hormone deficiencies (MPHD) including GH, as well as primary IGE1 deficiency (GH resistance, Laron syndrome) present signs of early ageing such as thin and wrinkled skin, obesity, hyperglycemia and osteoporosis. These changes do not seem to affect the lifespan, as patients reach old age. Animal models of genetic MPHD (Ames and Snell mice) and GH receptor knockout mice (primary IGF1 deficiency) also have a statistically significant higher longevity compared to normal controls. On the contrary, mice transgenic for GH and acromegalic patients secreting large amounts of GH have premature death. In conclusion longstanding GH/IGF1 deficiency affects several parameters of the ageing process without impairing lifespan, and as shown in animal models prolongs longevity. In contrast high GH/IGF1 levels accelerate death.

  17. Combined deficiency of factor V and factor VIII is due to mutations in either LMAN1 or MCFD2

    Science.gov (United States)

    Zhang, Bin; McGee, Beth; Yamaoka, Jennifer S.; Guglielmone, Hugo; Downes, Katharine A.; Minoldo, Salvador; Jarchum, Gustavo; Peyvandi, Flora; de Bosch, Norma B.; Ruiz-Saez, Arlette; Chatelain, Bernard; Olpinski, Marian; Bockenstedt, Paula; Sperl, Wolfgang; Kaufman, Randal J.; Nichols, William C.; Tuddenham, Edward G. D.; Ginsburg, David

    2006-01-01

    Mutations in LMAN1 (ERGIC-53) or MCFD2 cause combined deficiency of factor V and factor VIII (F5F8D). LMAN1 and MCFD2 form a protein complex that functions as a cargo receptor ferrying FV and FVIII from the endoplasmic reticulum to the Golgi. In this study, we analyzed 10 previously reported and 10 new F5F8D families. Mutations in the LMAN1 or MCFD2 genes accounted for 15 of these families, including 3 alleles resulting in no LMAN1 mRNA accumulation. Combined with our previous reports, we have identified LMAN1 or MCFD2 mutations as the causes of F5F8D in 71 of 76 families. Among the 5 families in which no mutations were identified, 3 were due to misdiagnosis, with the remaining 2 likely carrying LMAN1 or MCFD2 mutations that were missed by direct sequencing. Our results suggest that mutations in LMAN1 and MCFD2 may account for all cases of F5F8D. Immunoprecipitation and Western blot analysis detected a low level of LMAN1-MCFD2 complex in lymphoblasts derived from patients with missense mutations in LMAN1 (C475R) or MCFD2 (I136T), suggesting that complete loss of the complex may not be required for clinically significant reduction in FV and FVIII. PMID:16304051

  18. Tactic "Tulgama" the Art of War the Mongols XIII century

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    Leonid A. Bobrov

    2015-12-01

    Full Text Available In this publication, based on a comprehensive analysis of the sources, considered one of the most important tactics of the Mongols of the XIII century, known under the Turkic name "Tulgama". The name of the maneuver is derived from the Turkic verb "talamak", meaning "to surround, to wrapped up, to turn, to whirl, to twist". It is established that the maneuver "Tulgama" was a special tactic, providing coverage flank (flanks of the enemy in the rear and the application of massed archery shot in his builds. Selected three varieties under consideration tactic: right flank or left flank and steam room (double "Tulgama". The most typical was right flanks "Tulgama" when doing the nomads, covered the left wing of the enemy with its right flank. This allowed the attacking archers to fire from the most convenient position (left-forward, left, and left-back. A characteristic feature of the Mongol tactical art was a steam room (double "Tulgama" when the troops of nomads covered both enemy's flank, concluding the enemy's army in the ring, then attacked her from all four sides. A similar technique could not only shoot down from the position, but almost completely destroy the enemy troops. For the most efficient use of maneuver "Tulgama" the Mongols made increasing use of expanded mesalamines system, with a powerful thrust forward flank groups. The concentration of the shock troops on the flanks (reinforced, including by weakening the Central shelf is dramatically different from the tactics of the Mongols from the traditional tactics of the Turks, whose best part was typically the center of the combat formations. However, direct confrontation of these two approaches during the military campaigns of the XIII century demonstrated the advantages of the Mongol tactical schemes. High efficiency of reception "Tulgama" led to its continued existence in the military art of the nomads.

  19. Fibroblast growth factor 8 deficiency compromises the functional response of the serotonergic system to stress.

    Directory of Open Access Journals (Sweden)

    Leah R Brooks

    Full Text Available Functionally heterogeneous populations of serotonergic neurons, located within the dorsal raphe nucleus (DR, play a role in stress-related behaviors and neuropsychiatric illnesses such as anxiety and depression. Abnormal development of these neurons may permanently alter their structure and connections, making the organism more susceptible to anxiety-related disorders. A factor that critically regulates the development of serotonergic neurons is fibroblast growth factor 8 (Fgf8. In this study, we used acute restraint stress followed by behavioral testing to examine whether Fgf8 signaling during development is important for establishing functional stress- and anxiety-related DR neurocircuits in adulthood. Wild-type and heterozygous male mice globally hypomorphic for Fgf8 were exposed to acute restraint stress and then tested for anxiety-like behavior on the elevated plus-maze. Further, we measured c-Fos immunostaining as a marker of serotonergic neuronal activation and tissue 5-hydroxyindoleacetic acid concentrations as a marker of serotonin functional output. Results showed that Fgf8 hypomorphs exhibited 1 an exaggerated response of DR anxiety-promoting circuits and 2 a blunted response of a DR panic-inhibiting circuit to stress, effects that together were associated with increased baseline anxiety-like behavior. Overall, our results provide a neural substrate upon which Fgf8 deficiency could affect stress response and support the hypothesis that developmental disruptions of serotonergic neurons affect their postnatal functional integrity.

  20. Risk factors for vitamin A deficiency among preschool aged children in Pohnpei, Federated States of Micronesia.

    Science.gov (United States)

    Yamamura, Carrie M; Sullivan, Kevin M; van der Haar, Frits; Auerbach, Steven B; Iohp, Kidsen K

    2004-02-01

    In 1993, the Department of Health of the Federated States of Micronesia (FSM) conducted a population-based stratified random survey among 355 children aged 24-48 months in Pohnpei, one of the four FSM States. The objective was to determine the prevalence, and explore risk factors for vitamin A deficiency (VAD). Trained field workers collected data from a range of demographic, dietary and socioeconomic variables related to the children. The serum retinol concentration was 19.4 +/- 7.5 microg/dl (mean +/- SD), and the VAD prevalence (serum retinol <20 microg/dl) 53.1 per cent. The significant independent risk factors, determined by logistic regression, were: mother's work at home, sibling <2 years older, rural household located on the main island, early weaning, and child anemia, controlling for pipe water and electricity in the household. If compared with a reference of apparently healthy children of similar age in the USA, the distribution of serum retinol among young Pohnpei children was shifted entirely to low levels. We conclude that eliminating the pervasive VAD problem in Pohnpei would require a multi-pronged tactical approach that combines dietary improvement strategies with the ongoing supplementation effort.

  1. Ghrelin Inhibition Restores Glucose Homeostasis in Hepatocyte Nuclear Factor-1α (MODY3)-Deficient Mice.

    Science.gov (United States)

    Brial, François; Lussier, Carine R; Belleville, Karine; Sarret, Philippe; Boudreau, François

    2015-09-01

    Hepatocyte nuclear factor-1α (HNF1α) is a transcription factor expressed in tissues of endoderm origin. Mutations in HNF1A are associated with maturity-onset diabetes of the young 3 (MODY3). Mice deficient for Hnf1α are hyperglycemic, with their pancreatic β-cells being defective in glucose-sensing insulin secretion. The specific mechanisms involved in this defect are unclear. Gut hormones control glucose homeostasis. Our objective was to explore whether changes in these hormones play a role in glucose homeostasis in the absence of Hnf1α. An increase in ghrelin gene transcript and a decrease in glucose-dependent insulinotropic polypeptide (GIP) gene transcripts were observed in the gut of Hnf1α-null mice. These changes correlated with an increase of ghrelin and a decrease of GIP-labeled cells. Ghrelin serological levels were significantly induced in Hnf1α-null mice. Paradoxically, GIP levels were also induced in these mice. Treatment of Hnf1α-null mice with a ghrelin antagonist led to a recovery of the diabetic symptoms. We conclude that upregulation of ghrelin in the absence of Hnf1α impairs insulin secretion and can be reversed by pharmacological inhibition of ghrelin/GHS-R interaction. These observations open up on future strategies to counteract ghrelin action in a program that could become beneficial in controlling non-insulin-dependent diabetes.

  2. Factor V and VIII combined deficiency: clinical perioperative management for tonsillectomy in a child.

    Science.gov (United States)

    Lanchon, R; Robin, F; Brissaud, O; Marro, M; Nouette-Gaulain, K

    2014-03-01

    Combined factors V (FV) and VIII (FVIII) deficiency is a rarely seen hereditary coagulation disease. Experience of its management in surgery with a high-risk of bleeding is rare. The interest of this case report is to propose a strategy of perioperative management for such a deficit, but also to recall that a careful preoperative anesthetic evaluation with questioning and physical examination permits to detect unsuspected coagulation disorders and to schedule the preventive treatment. The protocol for the perioperative period consisted of the administration of desmopressin and fresh frozen plasma one hour before surgery. The administration of desmopressin was continued for 48hours. Fresh frozen plasma and tranexamic acid were administered during the first 9 postoperative days. A local bleeding occurred at 8 days (scab coming off) and required systematically a surgical hemostasis and an intensification of the therapeutic protocol. Recombinant plasmatic factor VIII was administered for 7 days together with a daily perfusion of fresh frozen plasma for a total treatment period of 14 days.

  3. Prevalence and factors associated with vitamin A deficiency in children and adolescents

    Directory of Open Access Journals (Sweden)

    Rita de Cássia Ribeiro-Silva

    2014-09-01

    Full Text Available Objective: To identify the prevalence and factors associated with vitamin A deficiency (VAD in children and adolescents. Methods: This was a cross-sectional study involving 546 schoolchildren, aged between 7 and 14 years, of both genders, enrolled in public elementary schools. Blood was collected for measurement of serum retinol. The retinol concentration in the samples was determined by high performance liquid chromatography (HPLC. Data were collected on anthropometrics, dietary, demographic, and socioeconomic factors. Polytomous logistic regression was used to evaluate the associations of interest. Results: Approximately 27.5% of the students had retinol values < 30 μg/dL. The multivariate analysis showed, after the appropriate adjustments, a positive and statistically significant association of moderate/severe VAD (OR = 2.19; 95% CI 1.17 to 4.10 and marginal VAD (OR = 2.34; 95% CI 1.47 to 3.73 with age < 10 years. There was also association of VAD moderate/severe (OR = 2.01; 95% CI 1.01 to 5.05 and borderline VAD (OR = 2.14; 95% CI: 1.08 to 4.21 with the anthropometric status of underweight. Lower intake of retinol was detected among those with severe VAD. Conclusion: VAD is a health concern among children and adolescents. Lower weight and younger schoolchildren had greater vulnerability to VAD.

  4. Neuroendocrine and Cardiovascular Risk Factors in Adults with Pituitary Growth Hormone Deficiency (Literature Review

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    S.I. Ismailov

    2013-06-01

    Full Text Available In this article authors discussed the results of literature review, which has been dedicated to study of different complications of growth hormone deficiency in adults, referring to the literature of the last 10–15 years. Based on this analysis, the authors concluded that in adults with growth hormone deficiency there is an adverse profile of cardiovascular risk. Patients with growth hormone deficiency have an adverse lipid profile, elevated body mass index, increased waist circumference and a high risk of hypertension. These disorders are likely to explain the increased cardiovascular mortality observed in patients with hypopituitarism, regardless of the etiology of growth hormone deficiency in adults.

  5. Coronary artery calcification in hemophilia A: No evidence for a protective effect of factor VIII deficiency on atherosclerosis

    NARCIS (Netherlands)

    Tuinenburg, A.; Rutten, A.; Kavousi, M.; Leebeek, F.W.G.; Ypma, P.F.; Laros-Van Gorkom, B.A.P.; Nijziel, M.R.; Kamphuisen, P.W.; Mauser-Bunschoten, E.P.; Roosendaal, G.; Biesma, D.H.; Van Der Lugt A., [No Value; Hofman, A.; Witteman, J.C.M.; Bots, M.L.; Schutgens, R.E.G.

    2011-01-01

    Mortality due to ischemic heart disease is lower in hemophilia patients when compared to the general male population. As coagulation plays a role in the inflammatory pathways involved in atherogenesis, we investigated whether the clotting factor deficiency protects hemophilia patients from developin

  6. Deficiency in milk fat globule-epidermal growth factor-factor 8 exacerbates organ injury and mortality in neonatal sepsis.

    Science.gov (United States)

    Hansen, Laura W; Khader, Adam; Yang, Weng-Lang; Jacob, Asha; Chen, Tracy; Nicastro, Jeffrey M; Coppa, Gene F; Prince, Jose M; Wang, Ping

    2017-09-01

    Neonatal sepsis is a systemic inflammation occurring in neonates because of a proven infection within the first 28days of birth. It is the third leading cause of morbidity and mortality in the newborns. The mechanism(s) underlying the systemic inflammation in neonatal sepsis has not been completely understood. We hypothesize that the deficiency of milk fat globule-epidermal growth factor-factor 8 (MFG-E8), a protein commonly found in human milk, could be responsible for the increased inflammatory response leading to morbidity and mortality in neonatal sepsis. Male and female newborn mice aged 5-7days were injected intraperitoneally with 0.9mg/g body weight cecal slurry (CS). At 10h after CS injection, they were euthanized, and blood, lungs and gut tissues were obtained for further analyses. Control newborn mice underwent similar procedures with the exception of the CS injection. In duplicate newborn mice after CS injection, they were returned to their respective cages with their mothers and were closely monitored for 7days and survival rate recorded. At 10h after CS injection, serum LDH in the MFG-E8 knockout (KO) newborn mice was significantly increased by 58% and serum IL-6, IL-1β and TNF-α in the MFG-E8KO newborn mice were also significantly increased by 56%, 65%, and 105%, respectively, from wild type (WT) newborn mice. There were no significant difference between WT control and MFG-E8 control newborn mice. The lung architecture was severely damaged and a significant 162% increase in injury score was observed in the CS MFG-E8KO newborn mice. The MPO, TUNEL staining, and cytokine levels in the lungs and the intestine in CS MFG-E8KO newborn mice were significantly increased from CS WT newborn mice. Similarly, intestinal integrity was also compromised in the CS MFG-E8KO newborn mice. In a survival study, while the mortality rate within 7days was only 29% in the CS WT newborn mice, 80% of the CS MFG-E8KO newborn mice died during the same time period with the

  7. El olvido de un sistema de cálculo de costes andalusí (primer tercio del siglo XIII

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    Jesús D. López Manjón

    2016-07-01

    Full Text Available Este trabajo da a conocer una parte del patrimonio histórico contable de Al-Andalus analizando los procedimientos de cálculo de costes incluidos en el Kitab fi Adab Al-Hisba (Libro de Buen Gobierno del Zoco, sus antecedentes y su posible transferencia a otros reinos. Dicho libro fue escrito en el primer tercio del siglo XIII por Al-Saqati. Estos cálculos se utilizaron para fijar precios al por menor. El estudio proporciona evidencia sobre el olvido de estos cálculos de costes en la Edad Media y analiza las causas de la no transmisión de este conocimiento a los reinos cristianos de la Península Ibérica. El trabajo pretende aportar tanto al conocimiento de la Historia de la Contabilidad en un contexto espacial y temporal poco estudiado como es Al-Andalus en el siglo XIII, como al de los mecanismos de transferencia de tecnologías contables. Se presenta un caso en el que los factores señalados en la literatura como inhibidores de dichos mecanismos inciden en la mayor dificultad para la transmisión de los conceptos contables.

  8. Gastric intrinsic factor deficiency with combined GIF heterozygous mutations and FUT2 secretor variant.

    Science.gov (United States)

    Chery, Celine; Hehn, Alain; Mrabet, Nadir; Oussalah, Abderrahim; Jeannesson, Elise; Besseau, Cyril; Alberto, Jean-Marc; Gross, Isabelle; Josse, Thomas; Gérard, Philippe; Guéant-Rodriguez, Rosa Maria; Freund, Jean-Noel; Devignes, Jean; Bourgaud, Frédérique; Peyrin-Biroulet, Laurent; Feillet, François; Guéant, Jean-Louis

    2013-05-01

    Several genome-wide association studies (GWAS) have identified a strong association between serum vitamin B12 and fucosyltransferase 2 (FUT2), a gene associated with susceptibility to Helicobacter pylori infection. Hazra et al. conducted a meta-analysis of three GWAS and found three additional loci in MUT, CUBN and TCN1. Other GWAS conducted in Italy and China confirmed the association for FUT2 gene. Alpha-2-fucosyltransferase (FUT2) catalyzes fucose addition to form H-type antigens in exocrine secretions. FUT2 non-secretor variant produces no secretion of H-type antigens and is associated with high-plasma vitamin B12 levels. This association was explained by the influence of FUT2 on H. pylori, which is a risk factor of gastritis, a main cause of vitamin B12 impaired absorption. However, we recently showed that H. pylori serology had no influence on FUT2 association with vitamin B12, in a large sample population, suggesting the involvement of an alternative mechanism. GIF is another gene associated with plasma levels of vitamin B12 and gastric intrinsic factor (GIF) is a fucosylated protein needed for B12 absorption. Inherited GIF deficiency produces B12 deficiency unrelated with gastritis. We report 2 families with heterozygous GIF mutation, 290T>C, M97T, with decreased binding affinity of GIF for vitamin B12 and one family with heterozygous GIF mutation 435_437delGAA, K145_N146delinsN and no B12 binding activity of mutated GIF. All cases with vitamin B12 deficit carried the FUT2 rs601338 secretor variant. Ulex europeus binding to GIF was influenced by FUT2 genotypes and GIF concentration was lower, in gastric juice from control subjects with the secretor genotype. GIF290C allele was reported in 5 European cases and no Africans among 1282 ambulatory subjects and was associated with low plasma vitamin B12 and anaemia in the single case bearing the FUT2 secretor variant. We concluded that FUT2 secretor variant worsens B12 status in cases with heterozygous GIF

  9. Chornobyl Accident and Iodine Deficiency as Risk Factors of Thyroid Pathology in Population of the Affected Regions of Ukraine

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    V.I. Кravchenko

    2016-04-01

    Full Text Available The result of the Chornobyl nuclear power plant accident was the negative effect of radiation on the health of population of large areas of Ukraine and surrounding countries, the impact of this action will be shown over the years. Studies conducted in the 2011–2014 in Kyiv, Zhytomyr and Chernihiv regions showed that iodine deficiency in the diet of the population in these areas remains and has a negative effect on the health of residents exposed to Chornobyl accident factors. Solution of the problem of iodine deficiency disorders prevention lies in the aspect of the adoption of legislation relating to the mass and group iodine prophylaxis.

  10. Fibroblast Growth Factor 21 Deficiency Attenuates Experimental Colitis-Induced Adipose Tissue Lipolysis

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    Liming Liu

    2017-01-01

    Full Text Available Aims. Nutrient deficiencies are common in patients with inflammatory bowel disease (IBD. Adipose tissue plays a critical role in regulating energy balance. Fibroblast growth factor 21 (FGF21 is an important endocrine metabolic regulator with emerging beneficial roles in lipid homeostasis. We investigated the impact of FGF21 in experimental colitis-induced epididymal white adipose tissue (eWAT lipolysis. Methods. Mice were given 2.5% dextran sulfate sodium (DSS ad libitum for 7 days to induce colitis. The role of FGF21 was investigated using antibody neutralization or knockout (KO mice. Lipolysis index and adipose lipolytic enzymes were determined. In addition, 3T3-L1 cells were pretreated with IL-6, followed by recombinant human FGF21 (rhFGF21 treatment; lipolysis was assessed. Results. DSS markedly decreased eWAT/body weight ratio and increased serum concentrations of free fatty acid (FFA and glycerol, indicating increased adipose tissue lipolysis. eWAT intracellular lipolytic enzyme expression/activation was significantly increased. These alterations were significantly attenuated in FGF21 KO mice and by circulating FGF21 neutralization. Moreover, DSS treatment markedly increased serum IL-6 and FGF21 levels. IL-6 pretreatment was necessary for the stimulatory effect of FGF21 on adipose lipolysis in 3T3-L1 cells. Conclusions. Our results demonstrate that experimental colitis induces eWAT lipolysis via an IL-6/FGF21-mediated signaling pathway.

  11. Eculizumab in neonatal hemolytic uremic syndrome with homozygous factor H deficiency.

    Science.gov (United States)

    Michaux, Katell; Bacchetta, Justine; Javouhey, Etienne; Cochat, Pierre; Frémaux-Bacchi, Véronique; Sellier-Leclerc, Anne-Laure

    2014-12-01

    Neonatal atypical hemolytic uremic syndrome (aHUS) is a rare but severe disease that is mainly due to methylmalonic aciduria or genetic complement abnormalities. Traditional management of aHUS includes plasma infusion/exchange, but in small or unstable infants, plasma exchange can be challenging because of high extracorporeal volume and difficulty to obtain an adequate venous access. The C5 complement blocker eculizumab has become a cornerstone of first-line management of aHUS due to complement deregulation in older patients. However, little data are available on its use in neonatal aHUS. We report on an 11-day-old neonate with severe aHUS (myocardial impairment, respiratory failure, acute kidney disease requiring hemodiafiltration) due to homozygous factor-H deficiency. She received early treatment with eculizumab as first-line therapy and completely recovered within 5 days. A second dose of eculizumab was administered 7 days after the first infusion, followed by a dose every 2 weeks for 2 months and then every 3 weeks, at the same dosage (300 mg). With more than 24 months of follow-up, renal function remains normal. We report on the long-term efficacy and safety of eculizumab as first-line therapy in neonatal aHUS. However its use still requires optimization in terms of indications and administration (frequency, dosage).

  12. Detection of Factor XI Deficiency (FXID and Complex Vertebral Malformation (CVM in Bali Cattle

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    S. W. Siswanti

    2014-12-01

    Full Text Available Factor XI Deficiency (FXID is caused by imperfect insertion of poly adenine which is resulted in introduction of premature stop codon in FXI gene. Substitution of guanine into thymine in SLC35A3 gene caused Complex Vertebral Malformation (CVM. The research was aimed to detect the presence or absence of a genetic defect mainly CVM using SLC35A3 gene and FXID using FXI gene in Indonesian Bali cattle. The presence of this genetic defect may have a significant economic impact on the breeding program. The research of genetic defect was done mostly in dairy cattle, but there was no report for screening of genetic defect in Bali cattle. In this study, 303 fresh blood samples and 22 semen samples which were collected from Indonesian Bali cattle breeding center (BPTU HMT Denpasar, BPT HMT Serading West Nusa Tenggara and district Barru South Sulawesi and artificial insemination centre (BBIB Singosari and BIBD Baturiti were used for screening of FXID and CVM. The amplicons of FXI gene were obtained by using PCR and that for SLC35A3 gene were obtained by using PCR-RFLP method with PstI restriction enzyme. These PCR products were analyzed by using 2% agarose gels electrophoresis. All genotypes were confirmed by DNA sequencing to determine an allele mutant. The allele mutant was not found in all of the samples. The result of this study showed that CVM and FXID were not detected in Bali cattle from Indonesian Bali cattle breeding and artificial insemination centres.

  13. The skeletal structure of insulin-like growth factor I-deficient mice

    Science.gov (United States)

    Bikle, D.; Majumdar, S.; Laib, A.; Powell-Braxton, L.; Rosen, C.; Beamer, W.; Nauman, E.; Leary, C.; Halloran, B.

    2001-01-01

    The importance of insulin-like growth factor I (IGF-I) for growth is well established. However, the lack of IGF-I on the skeleton has not been examined thoroughly. Therefore, we analyzed the structural properties of bone from mice rendered IGF-I deficient by homologous recombination (knockout [k/o]) using histomorphometry, peripheral quantitative computerized tomography (pQCT), and microcomputerized tomography (muCT). The k/o mice were 24% the size of their wild-type littermates at the time of study (4 months). The k/o tibias were 28% and L1 vertebrae were 26% the size of wild-type bones. Bone formation rates (BFR) of k/o tibias were 27% that of the wild-type littermates. The k/o bones responded normally to growth hormone (GH; 1.7-fold increase) and supranormally to IGF-I (5.2-fold increase) with respect to BFR. Cortical thickness of the proximal tibia was reduced 17% in the k/o mouse. However, trabecular bone volume (bone volume/total volume [BV/TV]) was increased 23% (male mice) and 88% (female mice) in the k/o mice compared with wild-type controls as a result of increased connectivity, increased number, and decreased spacing of the trabeculae. These changes were either less or not found in L1. Thus, lack of IGF-I leads to the development of a bone structure, which, although smaller, appears more compact.

  14. Heat shock transcription factor 1-deficiency attenuates overloading-associated hypertrophy of mouse soleus muscle.

    Science.gov (United States)

    Koya, Tomoyuki; Nishizawa, Sono; Ohno, Yoshitaka; Goto, Ayumi; Ikuta, Akihiro; Suzuki, Miho; Ohira, Tomotaka; Egawa, Tatsuro; Nakai, Akira; Sugiura, Takao; Ohira, Yoshinobu; Yoshioka, Toshitada; Beppu, Moroe; Goto, Katsumasa

    2013-01-01

    Hypertrophic stimuli, such as mechanical stress and overloading, induce stress response, which is mediated by heat shock transcription factor 1 (HSF1), and up-regulate heat shock proteins (HSPs) in mammalian skeletal muscles. Therefore, HSF1-associated stress response may play a key role in loading-associated skeletal muscle hypertrophy. The purpose of this study was to investigate the effects of HSF1-deficiency on skeletal muscle hypertrophy caused by overloading. Functional overloading on the left soleus was performed by cutting the distal tendons of gastrocnemius and plantaris muscles for 4 weeks. The right muscle served as the control. Soleus muscles from both hindlimbs were dissected 2 and 4 weeks after the operation. Hypertrophy of soleus muscle in HSF1-null mice was partially inhibited, compared with that in wild-type (C57BL/6J) mice. Absence of HSF1 partially attenuated the increase of muscle wet weight and fiber cross-sectional area of overloaded soleus muscle. Population of Pax7-positive muscle satellite cells in HSF1-null mice was significantly less than that in wild-type mice following 2 weeks of overloading (pmuscle hypertrophy might be attributed to the greater and prolonged enhancement of IL-6 expression. HSF1 and/or HSF1-mediated stress response may, in part, play a key role in loading-induced skeletal muscle hypertrophy.

  15. Vitamin B12 deficiency with intrinsic factor antibodies in an infant with poor growth and developmental delay.

    Science.gov (United States)

    McNeil, Kathleen; Chowdhury, Dhiman; Penney, Lynette; Rashid, Mohsin

    2014-02-01

    Vitamin B12 deficiency is very rare in infants and may lead to serious hematological and neurodevelopmental abnormalities. The present article describes a case involving a seven-month-old boy with severe vitamin B12 deficiency, likely caused by juvenile pernicious anemia, an entity rarely described. The child presented with feeding intolerance, poor growth and developmental delay. He was noted to have macrocytic anemia, a markedly low serum vitamin B12 level, and elevated homocysteine and methylmalonic acid levels. Antibodies to intrinsic factor were positive. The mother was healthy, with normal vitamin B12 status. Therapy with vitamin B12 supplements led to excellent recovery of symptoms. Vitamin B12 deficiency should be considered in children presenting with failure to thrive, especially when compounded with neurological symptoms. Early diagnosis and adequate treatment is essential to avoid serious complications.

  16. Do deficiencies in growth hormone and insulin-like growth factor-1 (IGF-1) shorten or prolong longevity?

    Science.gov (United States)

    Laron, Zvi

    2005-02-01

    Present knowledge on the effects of growth hormone (GH) and insulin-like growth factor-I (IGF-I) deficiency on aging and lifespan are controversial. Studying untreated patients with either isolated GH deficiency due to GH gene deletion, patients with multiple pituitary hormone deficiency due to PROP-1 gene mutation and patients with isolated IGF-I deficiency due to deletions or mutations of the GH receptor gene (Laron syndrome); it was found, that these patients despite signs of early aging (wrinkled skin, obesity, insulin resistance and osteopenia) have a long life span reaching ages of 80-90 years. Animal models of genetic GH deficiencies such as Snell mice (Pit-1 gene mutations) the Ames mice (PROP-1 gene mutation) and the Laron mice (GH receptor gene knock-out) have a statistically significant higher longevity compared to normal controls. On the contrary, mice transgenic for GH and acromegalic patients secreting high amounts of GH have premature death. Those data raise the question whether pharmacological GH administration to adults is deleterious, in contrast to policies advocating such therapies.

  17. Cell-wall-deficient bacteria: a major etiological factor for psoriasis?

    Institute of Scientific and Technical Information of China (English)

    WANG Guo-li; LI Xiu-yun; WANG Ming-yi; XIAO De-gui; ZHANG Yong-yu; YUAN Xiao-yan; WANG Qi-you; SONG Jian-jing

    2009-01-01

    Background Psoriasis is a common inflammatory skin disease, yet knowledge of the factors that may induce, trigger, or exacerbate psoriasis is not fully delineated. Recent advances have improved our understanding of the link between psoriasis and cell-wall-deficient bacteria (CWDB) infections. In the present study we assessed the prevalence of CWDB infection in patients with psoriasis.Methods The carriage rate of CWDB in the tonsil or pharynx of psoriasis patients, chronic tonsillitis patients and controls were investigated using hypertonic medium. Psoriasis patients with CWDB were randomly assigned to two groups and respectively treated with antibiotics or systemic therapy without antibiotic. Human peripheral blood mononuclear cells (PBMC) from psoriasis patients, chronic tonsillitis patients and control subjects were stimulated with bacteria antigens and extra-cellular levels of interferon-γ (IFN-γ) and interleukin (IL)-10 were measured in the supernatants using the ELISA technique, in vitro. Meanwhile, the proliferation ability of PBMC to respond to bacteria antigens was detected by MTT assay.Results CWDB were isolated from 74.2% of psoriasis patients, 23.5% of chronic tonsillitis patients and only 6.3% of controls. Antibiotic therapy was appropriate for approximately 80% of psoriasis patients with CWDB infection, and in only 8.9% psoriasis patients CWDB infection was detected after antibiotic therapy. Meanwhile, our study showed that CWDB and wide-type bacteria did remarkably enhance the production of IFN-γ, in vitro, and PBMC proliferation. Conclusion CWDB infection may be a virtual triggering factor in psoriasis by regulating T-cell activation.

  18. Comparable levels of activity and antigen in factor XII deficiency: a study of 21 homozygotes and 58 heterozygotes.

    Science.gov (United States)

    Girolami, A; Gavasso, S; Pacquola, E; Cabrio, L; Lombardi, A M; Girolami, B

    2005-07-01

    Results of coagulation studies on 21 homozygote patients with factor XII (FXII) deficiency revealed that all of them had no cross-reacting material (CRM) in their plasma. The 58 heterozygotes had in every instance an antigen level comparable to that of clotting activity namely, approximately 50% of normal. An analysis of all pertinent literature also showed that the presence of CRM is very rare in FXII deficiency. CRM is present in approximately 5% of homozygote patients. More precisely, seven of 145 patients. Only in one case, the antigen level was normal (FXII Washington). This prevalence appears lower than that observed for another contact phase factor (prekallikrein). The significance of blood abnormal forms of FXII has not been completely clarified yet. Their study appears useful in the attempt of clarifying the structure-function relation of factor XII.

  19. Identification of Allele Frequency of Factor XI Deficiency (FXID in Holstein Cows Reared in Thrace Region of Turkey

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    Kozet AVANUS

    2016-08-01

    Full Text Available Factor XI (FXI is a protein that plays a key role in plasma coagulation. Factor XI Deficiency (FXID is an autosomal recessive disease caused by an insertion into exon 12 of FXI gene. The aim of this study is to determine the allele frequency of Factor XI Deficiency (FXID in Holstein cows reared in Thrace region of Turkey. Blood samples of 287 Holstein cows were used for DNA isolation. Amplification of FXI gene was followed by the evaluation of PCR products with visualization on 2% agarose gel electrophoresis. FXID mutant allele was not observed in any of the samples used in this study. In conclusion, none of the Holstein cows were neither affected nor carriers for FXID among all analysed Holstein cows reared in Thrace region of Turkey.

  20. Cobalamin deficiency-induced changes of epidermal growth factor (EGF)-receptor expression and EGF levels in rat spinal cord.

    Science.gov (United States)

    Mutti, Elena; Magnaghi, Valerio; Veber, Daniela; Faroni, Alessandro; Pece, Salvatore; Di Fiore, Pier Paolo; Scalabrino, Giuseppe

    2011-02-28

    We investigated the effect of cobalamin (Cbl) deficiency on epidermal growth factor receptor (EGFR) mRNA levels in the spinal cord (SC) and liver of rats made Cbl-deficient (Cbl-D) by means of total gastrectomy or a Cbl-D diet, and simultaneously measured the levels of the epidermal growth factor (EGF). Both methods of inducing Cbl deficiency decreased EGFR expression in the SC and liver. Cbl replacement treatment normalized or nearly so most of the abnormalities in EGFR expression in the totally gastrectomized (TGX) rats at different times. The EGFR-immunostaining intensity decreased in the SC white matter of the Cbl-D rats and significantly increased in that of the TGX, Cbl-treated rats. EGF levels significantly increased in liver of TGX rats and in SC of 4-month TGX rats, and the increases returned to almost normal levels after a postoperative 2-month administration of Cbl to TGX rats. These findings demonstrate that Cbl deficiency dysregulates the EGFR-EGF dyad in these tissues.

  1. Prevalence of vitamin D deficiency its related factors in AqQala city in 2016

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    Abdolghader Taene

    2017-08-01

    Conclusion: Vitamin D is highly deficient in Agh Ghala inhabitants; particularly in men. This requires transferring the necessary information to the community and persuading them  to improve their life style.

  2. A potential screening factor for accumulation of cholesteyl ester transfer protein deficiency in East Asia: Schistosoma japonicum.

    Science.gov (United States)

    Yokoyama, Shinji

    2014-04-04

    Cholesteryl ester transfer protein (CETP)-deficiency manifests a unique plasma lipoprotein profile without other apparent symptoms. It is highly common in East Asia while rather rare anywhere else. A potential environmental screening factor(s) may therefore contribute to this eccentric distribution, such as its selective advantage against a regional illness, most likely an infectious disease, in relation to plasma lipoproteins. Blood flukes use the host plasma lipoproteins as nutrient sources through the lipoprotein receptor-like systems. Its Asian-specific species, Schistosoma (S) japonicum, which has been endemic in East Asia, takes up cholesteryl ester (CE) from high-density lipoprotein (HDL) for the embryonation of their eggs to miracidia, a critical step of the hepatic pathogenesis of this parasite, but poorly from HDL of CETP-deficiency. CD36-related protein (CD36RP) was cloned from the adults and the eggs of S. japonicum, with 1880-bp encoding 506 amino-acid residues exhibiting the CD36 domains and two transmembrane regions. Its extracellular domain selectively bound human HDL but neither LDL nor CETP-deficiency HDL, and the antibody against the extracellular domain suppressed the selective HDL-CE uptake and embryonation of the eggs. When infected with S. japonicum, wild-type mice developed less hepatic granulomatosis than CETP-transgenic mice by the ectopic egg embryonation. CD36RP is thus a candidate receptor of S. japonicum to facilitate uptake of HDL-CE necessary for egg embryonation. Abnormal HDL caused by CETP-deficiency retards this process and thereby protects the patients from development of hepatic lesions. S. japonicum infection is a potential screening factor for high prevalence of CETP deficiency in East Asia. Copyright © 2013 Elsevier B.V. All rights reserved.

  3. Is Cholesterol Sulfate Deficiency a Common Factor in Preeclampsia, Autism, and Pernicious Anemia?

    Directory of Open Access Journals (Sweden)

    Jingjing Liu

    2012-11-01

    Full Text Available In a recent paper, we proposed that a contributing factor in autism is a deficiency in cholesterol sulfate supply. In this paper, we investigate a link between preeclampsia and subsequent autism in the child, and we hypothesize that both conditions can be attributed to a severe depletion of cholesterol sulfate. Through studies on the Vaccine Adverse Event Reporting System (VAERS database, we demonstrate a strong statistical relationship among the signs and symptoms associated with autism and those associated with preeclampsia, pernicious anemia, and serious adverse reactions to vaccines. We show that VAERS reports associated with symptoms typical of pernicious anemia produce both a set of symptoms that are highly correlated with preeclampsia and another set highly correlated with autism. We explain this observation via an argument that, in a severe reaction, the cascade of events subsequent to vaccination reflects a profuse production of nitric oxide (NO and consequential destruction of both red blood cells (RBCs and cobalamin. This may explain the diverse signs and symptoms associated with both preeclampsia and severe vaccine adverse reactions. We argue that excess NO synthesis, induced by the aluminum and antigen in vaccines, results in hemolysis of RBCs, which allows hemoglobin to scavenge the excess NO, converting it to nitrate. The NO is also scavenged by cobalamin, leading to its inactivation and contributing to subsequent pernicious anemia. Finally, we demonstrate that severe adverse reactions to vaccines can be associated with life-threatening conditions related to the heart and brain, as well as stillbirth, when the vaccine is administered to a woman in the third-trimester of pregnancy, as demonstrated by statistical analysis of the Gardasil records.

  4. Disruption of the Suprachiasmatic Nucleus in Fibroblast Growth Factor Signaling-Deficient Mice.

    Science.gov (United States)

    Miller, Ann V; Kavanaugh, Scott I; Tsai, Pei-San

    2016-01-01

    Fibroblast growth factor (Fgf) 8 is essential for the development of multiple brain regions. Previous studies from our laboratory showed that reduced Fgf8 signaling led to the developmental alterations of neuroendocrine nuclei that originated within the diencephalon, including the paraventricular (PVN) and supraoptic (SON) nuclei. To further understand the role of Fgf8 in the development of other hypothalamic nuclei, we examined if Fgf8 and its cognate receptor, Fgfr1, also impact the integrity of the suprachiasmatic nuclei (SCN). The SCN control an organism's circadian rhythm and contain vasoactive intestinal peptide (VIP)-producing neurons as the main input neurons. Mice hypomorphic for Fgf8, Fgfr1, or both were examined for their SCN volume and the number of VIP neurons on postnatal day (PN) 0; adult hypomorphic mice were further examined for SCN function by quantifying SCN neuronal activation using cFos as a marker. On PN0, mice homozygous for Fgf8 hypomorphy displayed the most severe reduction of the SCN volume and VIP neurons. Those heterozygous for Fgf8 hypomorphy alone or Fgf8 combined with Fgfr1 hypomorphy, called double heterozygotes (DH), showed normal SCN volume but significantly reduced VIP neurons, albeit less severely than the homozygotes. Adult wild type, heterozygous Fgf8 hypomorphs (F8 Het), and DH mice were also examined for SCN cFos activation at three time points: 1 h (morning), 6 h (afternoon), and 11 h (evening) after light onset. In F8 Het mice, a significant change in the pattern of cFos immunostaining that may reflect delayed morning SCN activation was observed. Overall, our studies provide evidence supporting that deficiencies in Fgf8 not only impact the structural integrity of the SCN but also the pattern of SCN activation in response to light.

  5. Epidemiologic evidence supporting the role of maternal vitamin D deficiency as a risk factor for the development of infantile autism.

    Science.gov (United States)

    Grant, William B; Soles, Connie M

    2009-07-01

    This study examines whether maternal vitamin D deficiency is a risk factor for infantile autism disease (IAD). We used epidemiologic data seasonal variation of birth rates and prevalence of IAD for cohorts born before 1985. For seven studies reporting spring-to-summer excess birth rates for IAD, the season progressed from broad near 30 degrees N latitude, spring/summer in midlatitudes, to winter at the highest latitude. Also, using data from 10 studies, we found a strong effective latitudinal (related to wintertime solar ultraviolet B radiation) increase in IAD prevalence. These findings are consistent with maternal vitamin D deficiency's being a risk factor for IAD, possibly by affecting fetal brain development as well as possibly by affecting maternal immune system status during pregnancy. Further investigation of this hypothesis is warranted.

  6. Fibroblast growth factor receptor 4 (FGFR4) deficiency improves insulin resistance and glucose metabolism under diet-induced obesity conditions.

    Science.gov (United States)

    Ge, Hongfei; Zhang, Jun; Gong, Yan; Gupte, Jamila; Ye, Jay; Weiszmann, Jennifer; Samayoa, Kim; Coberly, Suzanne; Gardner, Jonitha; Wang, Huilan; Corbin, Tim; Chui, Danny; Baribault, Helene; Li, Yang

    2014-10-31

    The role of fibroblast growth factor receptor 4 (FGFR4) in regulating bile acid synthesis has been well defined; however, its reported role on glucose and energy metabolism remains unresolved. Here, we show that FGFR4 deficiency in mice leads to improvement in glucose metabolism, insulin sensitivity, and reduction in body weight under high fat conditions. Mechanism of action studies in FGFR4-deficient mice suggest that the effects are mediated in part by increased plasma levels of adiponectin and the endocrine FGF factors FGF21 and FGF15, the latter of which increase in response to an elevated bile acid pool. Direct actions of increased bile acids on bile acid receptors, and other potential indirect mechanisms, may also contribute to the observed metabolic changes. The results described herein suggest that FGFR4 antagonists alone, or in combination with other agents, could serve as a novel treatment for diabetes.

  7. Plague Epidemic s in Syria b etween XIII - XV. Centuries

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    Esra ATMACA

    2015-07-01

    Full Text Available Epidemic diseases that cause mass death has been one of the greatest fears of the society in the past century Usually due to poor living conditions, poverty, the inadequate treatment. Plague is one of them. Plague word is sometimes used synonymously with t he word tâûn, sometimes considered to be a greater sense of the Word plague. These outbreaks occured repeatedly in human society and many times occured between XIII - XV. centuries. Our research aims to examine the plague occured in Syria in the Mamluk state domination discussed period. One of the outbreaks have occured in the period between the years 1347 - 1351. Epidemic was looming at the same time with the European named the black death or large extinction. Many people have been killed in Syria as in other places where the epidemic has spread. Rumors about them are given in the source is situated in the form of the issuance of the number of people who died in one day and sometimes the total number of deaths took place at a given date range. In this study, we aimed to determine which is more severe than the others in the outbreak, to assess the rumor about the number of deaths from this cause, to reveal the difficulties of the funeral of the dead, to uncover practices that people do to get rid of this disease.

  8. Management of Pregnancy in a Chilean Patient with Congenital Deficiency of Factor VII and Glanzmann’s Thrombasthenia Variant

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    Nigel P. Murray

    2014-01-01

    Full Text Available Patients with inherited bleeding disorders are rare in obstetric practice but present with prolonged bleeding even after minor invasive procedures. They require a combined approach with obstetric and hematological management of each case, including the neonatal management of a possibly affected fetus. We present the case of a pregnancy in a patient with combined Factor VII deficiency and Glanzmann’s thrombasthenia, the successful obstetric and hematological management of the case, and a review of the literature.

  9. Chronic iron deficiency as an emerging risk factor for osteoporosis: a hypothesis.

    Science.gov (United States)

    Toxqui, Laura; Vaquero, M Pilar

    2015-04-02

    Iron is essential in oxygen transport and participates in many enzymatic systems in the body, with important roles in collagen synthesis and vitamin D metabolism. The relationship between iron and bone health comes from clinical observations in iron overload patients who suffered bone loss. The opposite scenario--whether iron deficiency, with or without anemia, affects bone metabolism--has not been fully addressed. This is of great interest, as this nutrient deficiency is a worldwide public health problem and at the same time osteoporosis and bone alterations are highly prevalent. This review presents current knowledge on nutritional iron deficiency and bone remodeling, the biomarkers to evaluate iron status and bone formation and resorption, and the link between iron and bone metabolism. Finally, it is hypothesized that chronic iron deficiency induces bone resorption and risk of osteoporosis, thus complete recovery from anemia and its prevention should be promoted in order to improve quality of life including bone health. Several mechanisms are suggested; hence, further investigation on the possible impact of chronic iron deficiency on the development of osteoporosis is needed.

  10. Cardiac deficiency of single cytochrome oxidase assembly factor scox induces p53-dependent apoptosis in a Drosophila cardiomyopathy model

    Science.gov (United States)

    Martínez-Morentin, Leticia; Martínez, Lidia; Piloto, Sarah; Yang, Hua; Schon, Eric A.; Garesse, Rafael; Bodmer, Rolf; Ocorr, Karen; Cervera, Margarita; Arredondo, Juan J.

    2015-01-01

    The heart is a muscle with high energy demands. Hence, most patients with mitochondrial disease produced by defects in the oxidative phosphorylation (OXPHOS) system are susceptible to cardiac involvement. The presentation of mitochondrial cardiomyopathy includes hypertrophic, dilated and left ventricular noncompaction, but the molecular mechanisms involved in cardiac impairment are unknown. One of the most frequent OXPHOS defects in humans frequently associated with cardiomyopathy is cytochrome c oxidase (COX) deficiency caused by mutations in COX assembly factors such as Sco1 and Sco2. To investigate the molecular mechanisms that underlie the cardiomyopathy associated with Sco deficiency, we have heart specifically interfered scox expression, the single Drosophila Sco orthologue. Cardiac-specific knockdown of scox reduces fly lifespan, and it severely compromises heart function and structure, producing dilated cardiomyopathy. Cardiomyocytes with low levels of scox have a significant reduction in COX activity and they undergo a metabolic switch from OXPHOS to glycolysis, mimicking the clinical features found in patients harbouring Sco mutations. The major cardiac defects observed are produced by a significant increase in apoptosis, which is dp53-dependent. Genetic and molecular evidence strongly suggest that dp53 is directly involved in the development of the cardiomyopathy induced by scox deficiency. Remarkably, apoptosis is enhanced in the muscle and liver of Sco2 knock-out mice, clearly suggesting that cell death is a key feature of the COX deficiencies produced by mutations in Sco genes in humans. PMID:25792727

  11. Risk Factors of Development of Iron-Deficiency Conditions in Moscow Adolescents

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    I. N. Zakharova

    2015-01-01

    Full Text Available The article presents data on prevalence structure and causes of iron-deficiency conditions (IDC in adolescents. The authors describe both literature data and the findings of their own study in the adolescents (n = 337 studying at Moscow comprehensive schools. Iron- deficiency anemia was revealed in 5.3% of the examined adolescents, latent iron deficiency — in 17%; vast majority of the last were females. The authors also determined the most common causes of IDC development in adolescents: growth spurt (according to the anamnesis, a source of chronic blood loss (prolonged and abundant menstruations [in girls], frequent nasal bleeding, vegetarianism, intense physical activity, diet compliance, excess weight, and obesity. 

  12. The clinical expression of hereditary protein C and protein S deficiency: : a relation to clinical thrombotic risk-factors and to levels of protein C and protein S

    NARCIS (Netherlands)

    Henkens, C. M. A.; van der Meer, J.; Hillege, J. L.; Bom, V. J. J.; Halie, M. R.; van der Schaaf, W.

    We investigated 103 first-degree relatives of 13 unrelated protein C or protein S deficient patients to assess the role of additional thrombotic risk factors and of protein C and protein S levels in the clinical expression of hereditary protein C and protein S deficiency. Fifty-seven relatives were

  13. Prevalence and associated factors of clinical manifestations of vitamin a deficiency among preschool children in asgede-tsimbla rural district, north Ethiopia, a community based cross sectional study

    OpenAIRE

    Abrha, Tesfalem; Girma, Yonas; Haile, Kebede; Hailu, Mezgebe; Hailemariam, Mengistu

    2016-01-01

    Background Vitamin A Deficiency is a common form of micronutrient deficiency, globally affecting 33.3 % of preschool-age children. An estimated of 44.4 % of preschool children in Africa were at risk for vitamin A deficiency. In Ethiopia, vitamin A deficiency leads to 80,000 deaths a year and affects 61 % of preschool children. The aim of this study was to investigate the prevalence and associated factors with the night blindness, Bitot’s spot and vitamin A intake among preschool children in r...

  14. Hydrolysed Formula Is a Risk Factor for Vitamin K Deficiency in Infants With Unrecognised Cholestasis

    NARCIS (Netherlands)

    van Hasselt, P. M.; de Vries, W.; de Vries, E.; Kok, K.; Cranenburg, E. C. M.; de Koning, T. J.; Schurgers, L. J.; Verkade, H. J.; Houwen, R. H. J.; Havinga, Rick

    2010-01-01

    Objectives: Vitamin K deficiency (VKD) may cause life-threatening haemorrhages, especially in breast-fed infants with unrecognised cholestasis. Interestingly, hypoallergenic formulas appear overrepresented in reported cases of VKD bleeding (VKDB) in formula-fed infants. We therefore assessed whether

  15. Is GERD a Factor in Osteonecrosis of the Jaw? Evidence of Pathology Linked to G6PD Deficiency and Sulfomucins

    Science.gov (United States)

    Swanson, Nancy L.; Li, Chen

    2016-01-01

    Osteonecrosis of the jaw (ONJ), a rare side effect of bisphosphonate therapy, is a debilitating disorder with a poorly understood etiology. FDA's Adverse Event Reporting System (FAERS) provides the opportunity to investigate this disease. Our goals were to analyze FAERS data to discover possible relationships between ONJ and specific conditions and drugs and then to consult the scientific literature to deduce biological explanations. Our methodology revealed a very strong association between gastroesophageal reflux and bisphosphonate-induced ONJ, suggesting acidosis as a key factor. Overgrowth of acidophilic species, particularly Streptococcus mutans, in the oral microbiome in the context of insufficient acid buffering due to impaired salivary glands maintains the low pH that sustains damage to the mucosa. Significant associations between ONJ and adrenal insufficiency, vitamin C deficiency, and Sjögren's syndrome were found. Glucose 6 phosphate dehydrogenase (G6PD) deficiency can explain much of the pathology. An inability to maintain vitamin C and other antioxidants in the reduced form leads to vascular oxidative damage and impaired adrenal function. Thus, pathogen-induced acidosis, hypoxia, and insufficient antioxidant defenses together induce ONJ. G6PD deficiency and adrenal insufficiency are underlying factors. Impaired supply of adrenal-derived sulfated sterols such as DHEA sulfate may drive the disease process.

  16. Is GERD a Factor in Osteonecrosis of the Jaw? Evidence of Pathology Linked to G6PD Deficiency and Sulfomucins

    Directory of Open Access Journals (Sweden)

    Stephanie Seneff

    2016-01-01

    Full Text Available Osteonecrosis of the jaw (ONJ, a rare side effect of bisphosphonate therapy, is a debilitating disorder with a poorly understood etiology. FDA’s Adverse Event Reporting System (FAERS provides the opportunity to investigate this disease. Our goals were to analyze FAERS data to discover possible relationships between ONJ and specific conditions and drugs and then to consult the scientific literature to deduce biological explanations. Our methodology revealed a very strong association between gastroesophageal reflux and bisphosphonate-induced ONJ, suggesting acidosis as a key factor. Overgrowth of acidophilic species, particularly Streptococcus mutans, in the oral microbiome in the context of insufficient acid buffering due to impaired salivary glands maintains the low pH that sustains damage to the mucosa. Significant associations between ONJ and adrenal insufficiency, vitamin C deficiency, and Sjögren’s syndrome were found. Glucose 6 phosphate dehydrogenase (G6PD deficiency can explain much of the pathology. An inability to maintain vitamin C and other antioxidants in the reduced form leads to vascular oxidative damage and impaired adrenal function. Thus, pathogen-induced acidosis, hypoxia, and insufficient antioxidant defenses together induce ONJ. G6PD deficiency and adrenal insufficiency are underlying factors. Impaired supply of adrenal-derived sulfated sterols such as DHEA sulfate may drive the disease process.

  17. Transcriptional profiling revealed the anti-proliferative effect of MFN2 deficiency and identified risk factors in lung adenocarcinoma.

    Science.gov (United States)

    Lou, Yuqing; Zhang, Yanwei; Li, Rong; Gu, Ping; Xiong, Liwen; Zhong, Hua; Zhang, Wei; Han, Baohui

    2016-07-01

    Mitofusin-2 (MFN2) was initially identified as a hyperplasia suppressor in hyper-proliferative vascular smooth muscle cells (VSMCs) of hypertensive rat arteries, which has also been implicated in various cancers. There exists a controversy in whether it is an oncogene or exerting anti-proliferative effect on tumor cells. Our previous cell cycle analysis and MTT assay showed that cell proliferation was inhibited in MFN2 deficient A549 human lung adenocarcinoma cells, without investigating the changes in regulatory network or addressing the underlying mechanisms. Here, we performed expression profiling in MFN2 knockdown A549 cells and found that cancer-related pathways were among the most susceptible pathways to MFN2 deficiency. Through comparison with expression profiling of a cohort consisting of 61 pairs of tumor-normal matched samples from The Cancer Genome Atlas (TCGA), we teased out the specific pathways to address the impact that MFN2 ablation had on A549 cells, as well as identified a few genes whose expression level associated with clinicopathologic parameters. In addition, transcriptional factor target enrichment analysis identified E2F as a potential transcription factor that was deregulated in response to MFN2 deficiency. Although bioinformatics analysis usually entail further verification, our study provided considerable information for future scientific inquiries in related areas as well as a paradigm for characterizing perturbation in regulatory network.

  18. Vitamin D receptor deficiency and low vitamin D diet stimulate aortic calcification and osteogenic key factor expression in mice.

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    Nadine Schmidt

    Full Text Available Low levels of 25-hydroxy vitamin D (25(OHD are associated with cardiovascular diseases. Herein, we tested the hypothesis that vitamin D deficiency could be a causal factor in atherosclerotic vascular changes and vascular calcification. Aortic root sections of vitamin D receptor knockout (VDR(-/- mice that were stained for vascular calcification and immunostained for osteoblastic differentiation factors showed more calcified areas and a higher expression of the osteogenic key factors Msx2, Bmp2, and Runx2 than the wild-type mice (P<0.01. Data from LDL receptor knockout (LDLR(-/- mice that were fed western diet with either low (50 IU/kg, recommended (1,000 IU/kg, or high (10,000 IU/kg amounts of vitamin D(3 over 16 weeks revealed increasing plasma concentrations of 25(OHD (P<0.001 with increasing intake of vitamin D, whereas levels of calcium and phosphorus in plasma and femur were not influenced by the dietary treatment. Mice treated with the low vitamin D diet had more calcified lesions and a higher expression of Msx2, Bmp2, and Runx2 in aortic roots than mice fed recommended or high amounts of vitamin D (P<0.001. Taken together, these findings indicate vitamin D deficiency as a risk factor for aortic valve and aortic vessel calcification and a stimulator of osteogenic key factor expression in these vascular areas.

  19. Roles of Group 2 Sigma Factors in Acclimation of the Cyanobacterium Synechocystis sp. PCC 6803 to Nitrogen Deficiency.

    Science.gov (United States)

    Antal, Taras; Kurkela, Juha; Parikainen, Marjaana; Kårlund, Anna; Hakkila, Kaisa; Tyystjärvi, Esa; Tyystjärvi, Taina

    2016-06-01

    Acclimation of cyanobacteria to environmental conditions is mainly controlled at the transcriptional level, and σ factors of the RNA polymerase have a central role in this process. The model cyanobacterium Synechocystis sp. PCC 6803 has four non-essential group 2 σ factors (SigB, SigC, SigD and SigE) that regulate global metabolic responses to various adverse environmental conditions. Here we show that although none of the group 2 σ factors is essential for the major metabolic realignments induced by a short period of nitrogen starvation, the quadruple mutant without any group 2 σ factors and triple mutants missing both SigB and SigD grow slowly in BG-11 medium containing only 5% of the nitrate present in standard BG-11. These ΔsigBCDE, ΔsigBCD and ΔsigBDE strains lost PSII activity rapidly in low nitrogen and accumulated less glycogen than the control strain. An abnormally high glycogen content was detected in ΔsigBCE (SigD is active), while the carotenoid content became high in ΔsigCDE (SigB is active), indicating that SigB and SigD regulate the partitioning of carbon skeletons in low nitrogen. Long-term survival and recovery of the cells after nitrogen deficiency was strongly dependent on group 2 σ factors. The quadruple mutant and the ΔsigBDE strain (only SigC is active) recovered more slowly from nitrogen deficiency than the control strain, and ΔsigBCDE in particular lost viability during nitrogen starvation. Nitrogen deficiency-induced changes in the pigment content of the control strain recovered essentially in 1 d in nitrogen-replete medium, but little recovery occurred in ΔsigBCDE and ΔsigBDE.

  20. DETECTION ON THE VAL34LEU POLYMORPHISM OF COAGULATION FACTOR XIII IN WOMEN WITH RECURRENT SPONTANEOUS ABORTION AND NORMAL WOMEN%反复自然流产妇女和正常妇女FXIII Val34Leu基因多态性的检测

    Institute of Scientific and Technical Information of China (English)

    霍梅; 林琳华; 黄明; 黄蓉; 朱莹; 何桂蓉; 唐署明; 方红辉; 李体远

    2008-01-01

    [目的]了解深圳地区反复自然流产妇女和正常妇女凝血因子XIII(FXIII)基因Val34Leu的多态性,探讨其与反复自然流产发病的关系.[方法]应用聚合酶链反应-限制性片段长度多态性分析方法(PCR-RFLP)检测57例反复自然流产妇女和153例正常妇女的FXIII Val34Leu基因多态性.[结果]反复自然流产组与正常对照组均不存在FXIII Val34Leu的突变.[结论]深圳地区正常妇女和反复自然流产妇女FXIII Val34Leu的突变频率很低.FXIII Val34Leu的突变可能不是引起反复自然流产发病的重要原因.

  1. A simple technique to reduce epistaxis and nasopharyngeal trauma during nasotracheal intubation in a child with factor IX deficiency having dental restoration.

    Science.gov (United States)

    Delgado, Anita V; Sanders, John C

    2004-10-01

    Epistaxis and airway trauma are often associated with nasotracheal intubation. We describe a patient with Factor IX deficiency who required nasotracheal intubation. An inexpensive, nonproprietary, rapid technique was used to reduce the trauma of intubation.

  2. Is vitamin d deficiency a new risk factor for cardiovascular disease?

    Science.gov (United States)

    Mandarino, Natália Ribeiro; Júnior, Francisco das Chagas Monteiro; Salgado, João Victor Leal; Lages, Joyce Santos; Filho, Natalino Salgado

    2015-01-01

    The role of vitamin D in the regulation of bone metabolism has been well established. However, in recent years, many studies have demonstrated that its role extends far beyond bone health. Growing evidence has shown a strong association between vitamin D deficiency and hypertension, metabolic syndrome, diabetes mellitus and atherosclerosis. The mechanisms by which vitamin D exerts its cardiovascular protective effects are still not completely understood, but there is evidence that it participates in the regulation of renin-angiotensin system and the mechanisms of insulin sensitivity and activity of inflammatory cytokines, besides its direct cardiovascular actions. In this review, several studies linking vitamin D deficiency with cardiometabolic risk as well as small randomized trials that have evaluated the cardiovascular effects of its supplementation are presented. However, large randomized placebo-controlled studies are still needed before we can definitively establish the role of vitamin D supplementation in the prevention and control of cardiovascular disease.

  3. The oxidative stress responsive transcription factor Pap1 confers DNA damage resistance on checkpoint-deficient fission yeast cells.

    Directory of Open Access Journals (Sweden)

    Carrie Belfield

    Full Text Available Eukaryotic cells invoke mechanisms to promote survival when confronted with cellular stress or damage to the genome. The protein kinase Chk1 is an integral and conserved component of the DNA damage response pathway. Mutation or inhibition of Chk1 results in mitotic death when cells are exposed to DNA damage. Oxidative stress activates a pathway that results in nuclear accumulation of the bZIP transcription factor Pap1. We report the novel finding that fission yeast Pap1 confers resistance to drug- and non-drug-induced DNA damage even when the DNA damage checkpoint is compromised. Multi-copy expression of Pap1 restores growth to chk1-deficient cells exposed to camptothecin or hydroxyurea. Unexpectedly, increased Pap1 expression also promotes survival of chk1-deficient cells with mutations in genes encoding DNA ligase (cdc17 or DNA polymerase δ (cdc6, but not DNA replication initiation mutants. The ability of Pap1 to confer resistance to DNA damage was not specific to chk1 mutants, as it also improved survival of rad1- and rad9-deficient cells in the presence of CPT. To confer resistance to DNA damage Pap1 must localize to the nucleus and be transcriptionally active.

  4. Proteomic analysis of the function of sigma factor σ54 in Helicobacter pylori survival with nutrition deficiency stress in vitro.

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    Yundong Sun

    Full Text Available H. pylori can survive under a nutrition-deficient environment. During infection and transmission, H. pylori is confronted with nutrient limitation and the bacterium requires rapid alteration in gene expression for survival under stress conditions. However, the mechanism underlining this regulation remains unknown. A previous study showed that σ(54 is an important regulation factor for H. pylori survival in the nutrition-deficient environment. Our results show that the expression of σ(54 (rpoN is significantly induced in the stationary phase (nutrition deficiency and the rpoN mutant showed a significantly lower viability than wild-type H. pylori in the late stationary phase. Thus, σ(54 is involved in H. pylori survival during nutrient limitation. We used comparative proteomics to analyze the protein differentiation between wild-type and rpoN mutant during the stationary phase. With depleted nutrients, σ(54 can slow the process of proliferation by negatively regulating genes involved in energy metabolism and biosynthesis and enhance stress-resistant ability by positively regulating genes involved in protein fate and redox reaction. Especially, NapA positively regulated by σ(54 plays an important function in H. pylori survival both in the stationary phase and in water, and the latter situation would be beneficial for bacterial in vitro transmission. Our investigations give new light on the adaptive regulation of H. pylori under stress conditions.

  5. Deficiency of the Complement Component 3 but Not Factor B Aggravates Staphylococcus aureus Septic Arthritis in Mice.

    Science.gov (United States)

    Na, Manli; Jarneborn, Anders; Ali, Abukar; Welin, Amanda; Magnusson, Malin; Stokowska, Anna; Pekna, Marcela; Jin, Tao

    2016-04-01

    The complement system plays an essential role in the innate immune response and protection against bacterial infections. However, detailed knowledge regarding the role of complement in Staphylococcus aureus septic arthritis is still largely missing. In this study, we elucidated the roles of selected complement proteins in S. aureus septic arthritis. Mice lacking the complement component 3 (C3(-/-)), complement factor B (fB(-/-)), and receptor for C3-derived anaphylatoxin C3a (C3aR(-/-)) and wild-type (WT) control mice were intravenously or intra-articularly inoculated with S. aureus strain Newman. The clinical course of septic arthritis, as well as histopathological and radiological changes in joints, was assessed. After intravenous inoculation, arthritis severity and frequency were significantly higher in C3(-/-)mice than in WT controls, whereas fB(-/-)mice displayed intermediate arthritis severity and frequency. This was in accordance with both histopathological and radiological findings. C3, but not fB, deficiency was associated with greater weight loss, more frequent kidney abscesses, and higher bacterial burden in kidneys. S. aureus opsonized with C3(-/-)sera displayed decreased uptake by mouse peritoneal macrophages compared with bacteria opsonized with WT or fB(-/-)sera. C3aR deficiency had no effect on the course of hematogenous S. aureus septic arthritis. We conclude that C3 deficiency increases susceptibility to hematogenous S. aureus septic arthritis and impairs host bacterial clearance, conceivably due to diminished opsonization and phagocytosis of S. aureus.

  6. Vitamin D Deficiency and Depressive Symptomatology in Psychiatric Patients Hospitalized with a Current Depressive Episode: A Factor Analytic Study.

    Directory of Open Access Journals (Sweden)

    Roland von Känel

    Full Text Available Low vitamin D levels have been associated with depressive symptoms in population-based studies and non-clinical samples as well as with clinical depression. This study aimed to examine the association of vitamin D levels with the severity and dimensions of depressive symptoms in hospitalized patients with a current episode of depression taking into account confounding variables.We investigated 380 patients (mean age 47 ± 12 years, 70% women who were consecutively hospitalized with a main diagnosis of an ICD-10 depressive episode. All patients self-rated depressive symptom severity with the Hospital Anxiety and Depression Scale (HADS-D, the Beck Depression Inventory-II (BDI-II, and the Brief Symptom Inventory. A principal component analysis was performed with all 34 items of these questionnaires and serum levels of 25-hydroxyvitamin D3 (25-OH D were measured.Vitamin D deficiency ( 75 nmol/l were present in 55.5%, 31.8% and 12.6%, respectively, of patients. Patients with vitamin D deficiency scored higher on the HADS-D scale and on an anhedonia symptom factor than those with insufficient (p-values ≤ 0.023 or sufficient (p-values ≤ 0.008 vitamin D. Vitamin D deficient patients also scored higher on the BDI-II scale than those with sufficient vitamin D (p = 0.007; BDI-II cognitive/affective symptoms, but not somatic/affective symptoms, were higher in patients with vitamin D deficiency (p = 0.005 and insufficiency (p = 0.041 relative to those with sufficient vitamin D. Effect sizes suggested clinically relevant findings.Low vitamin D levels are frequent in hospitalized patients with a current episode of depression. Especially 25-OH D levels < 50 nmol/l were associated with cognitive/affective depressive symptoms, and anhedonia symptoms in particular.

  7. Vitamin D Deficiency and Depressive Symptomatology in Psychiatric Patients Hospitalized with a Current Depressive Episode: A Factor Analytic Study.

    Science.gov (United States)

    von Känel, Roland; Fardad, Nasser; Steurer, Nadine; Horak, Nicole; Hindermann, Esther; Fischer, Franz; Gessler, Katharina

    2015-01-01

    Low vitamin D levels have been associated with depressive symptoms in population-based studies and non-clinical samples as well as with clinical depression. This study aimed to examine the association of vitamin D levels with the severity and dimensions of depressive symptoms in hospitalized patients with a current episode of depression taking into account confounding variables. We investigated 380 patients (mean age 47 ± 12 years, 70% women) who were consecutively hospitalized with a main diagnosis of an ICD-10 depressive episode. All patients self-rated depressive symptom severity with the Hospital Anxiety and Depression Scale (HADS-D), the Beck Depression Inventory-II (BDI-II), and the Brief Symptom Inventory. A principal component analysis was performed with all 34 items of these questionnaires and serum levels of 25-hydroxyvitamin D3 (25-OH D) were measured. Vitamin D deficiency ( 75 nmol/l) were present in 55.5%, 31.8% and 12.6%, respectively, of patients. Patients with vitamin D deficiency scored higher on the HADS-D scale and on an anhedonia symptom factor than those with insufficient (p-values ≤ 0.023) or sufficient (p-values ≤ 0.008) vitamin D. Vitamin D deficient patients also scored higher on the BDI-II scale than those with sufficient vitamin D (p = 0.007); BDI-II cognitive/affective symptoms, but not somatic/affective symptoms, were higher in patients with vitamin D deficiency (p = 0.005) and insufficiency (p = 0.041) relative to those with sufficient vitamin D. Effect sizes suggested clinically relevant findings. Low vitamin D levels are frequent in hospitalized patients with a current episode of depression. Especially 25-OH D levels < 50 nmol/l were associated with cognitive/affective depressive symptoms, and anhedonia symptoms in particular.

  8. EDITORIAL: XIII Mexican Workshop on Particles and Fields

    Science.gov (United States)

    Barranco, Juan; Contreras, Guillermo; Delepine, David; Napsuciale, Mauro

    2012-08-01

    Juan Barranco Physics Department, Guanajuato University, Loma del Bosque 103, col. Loma del Campestre, 37150, Leon (Mexico) jbarranc@fisica.ugto.mx Guillermo Contreras Departamento de Fisica Aplicada Centro de Investigacion y de Estudios Avanzados del Instituto Politecnico Nacional, Merida (Mexico) jgcn@mda.cinvestav.mx David Delepine Physics Department, Guanajuato University, Loma del Bosque 103, col. Loma del Campestre, 37150, Leon (Mexico) delepine@fisica.ugto.mx Mauro Napsuciale Physics Department, Guanajuato University, Loma del Bosque 103, col. Loma del Campestre, 37150, Leon (Mexico) mauro@fisica.ugto.mx The XIII Mexican Workshop on Particles and Fields (MWPF) took place from 20-26 October 2011, in the city of León, Guanajuato, México. This is a biennial meeting organized by the Division of Particles and Fields of the Mexican Physical Society designed to gather specialists in different areas of high energy physics to discuss the latest developments in the field. The thirteenth edition of this meeting was hosted by the Department of Cultural Studies of Guanajuato University in a nice environment dedicated to the Arts and Culture. The XIII MWPF was organized by three working groups who organized the corresponding sessions around three topics. The first one was Strings, Cosmology, Astroparticles and Physics Beyond the Standard Model. In this category we included: Cosmic Rays, Gamma Ray Bursts, Physics Beyond the Standard Model (theory and experimental searches), Strings and Cosmology. The working group for this topic was formed by Arnulfo Zepeda, Oscar Loaiza, Axel de la Macorra and Myriam Mondragón. The second topic was Hadronic Matter which included Perturbative QCD, Jets and Diffractive Physics, Hadronic Structure, Soft QCD, Hadron Spectroscopy, Heavy Ion Collisions and Soft Physics at Hadron Colliders, Lattice Results and Instrumentation. The working group for this topic was integrated by Wolfgang Bietenholz and Mariana Kirchbach. The third topic was

  9. 25 CFR 36.40 - Standard XIII-Library/media program.

    Science.gov (United States)

    2010-04-01

    ... 25 Indians 1 2010-04-01 2010-04-01 false Standard XIII-Library/media program. 36.40 Section 36.40 Indians BUREAU OF INDIAN AFFAIRS, DEPARTMENT OF THE INTERIOR EDUCATION MINIMUM ACADEMIC STANDARDS FOR THE... § 36.40 Standard XIII—Library/media program. (a) Each school shall provide a library/media...

  10. Inherited glutathione-S-transferase deficiency is a risk factor for pulmonary asbestosis.

    Science.gov (United States)

    Smith, C M; Kelsey, K T; Wiencke, J K; Leyden, K; Levin, S; Christiani, D C

    1994-09-01

    Pulmonary diseases attributable to asbestos exposure constitute a significant public health burden, yet few studies have investigated potential genetic determinants of susceptibility to asbestos-related diseases. The glutathione-S-transferases are a family of conjugating enzymes that both catalyze the detoxification of a variety of potentially cytotoxic electrophilic agents and act in the generation of sulfadipeptide leukotriene inflammatory mediators. The gene encoding glutathione-S-transferase class mu (GSTM-1) is polymorphic; approximately 50% of Caucasian individuals have a homozygous deletion of this gene and do not produce functional enzyme. Glutathione-S-transferase mu (GST-mu) deficiency has been previously reported to be associated with smoking-induced lung cancer. We conducted a cross-sectional study to examine the prevalence of the homozygous deletion for the GSTM-1 gene in members of the carpentry trade occupationally exposed to asbestos. Members of the United Brotherhood of Carpenters and Joiners of America attending their 1991 National Union conference were invited to participate. Each participant was offered a chest X-ray and was asked to complete a comprehensive questionnaire and have their blood drawn. All radiographs were assessed for the presence of pneumoconiosis in a blinded fashion by a National Institute for Occupational Safety and Health-certified International Labor Office "B" reader. Individual GSTM-1 status was determined using polymerase chain reaction methods. Six hundred fifty-eight workers were studied. Of these, 80 (12.2%) had X-ray abnormalities associated with asbestos exposure. Individuals genetically deficient in GST-mu were significantly more likely to have radiographic evidence of nonmalignant asbestos-related disease than those who were not deficient (chi 2 = 5.0; P < 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)

  11. HEREDITARY FACTOR VII DEFICIENCY IN THE ASIAN ELEPHANT (ELEPHAS MAXIMUS) CAUSED BY A F7 MISSENSE MUTATION.

    Science.gov (United States)

    Lynch, Michael; McGrath, Ken; Raj, Karthik; McLaren, Philippa; Payne, Karen; McCoy, Richard; Giger, Urs

    2017-04-01

    Hereditary disorders and genetic predispositions to disease are rarely reported in captive and free-ranging wildlife, and none have been definitively identified and characterized in elephants. A wild-caught, 41-yr-old male Asian elephant ( Elephas maximus ) without an apparent increased bleeding tendency was consistently found to have prolonged prothrombin times (PTs, mean=55±35 s) compared to 17 other elephants (PT=10±2 s). This elephant's partial thromboplastin times (PTT) fell within the normal range of the other elephants (12-30 s). A prolonged PT in the presence of a normal PTT suggests disruption of the extrinsic pathway via deficiency of coagulation Factor VII (FVII). This elephant's plasma FVII activity was very low (2%) compared to that of 15 other elephants (57-80%), but other coagulation factors' activities did not differ from the control elephants. Sequencing of genomic DNA from ethylenediaminetetraacetic acid blood revealed a single homozygous point mutation (c.202A>G) in the F7 gene of the FVII deficient elephant that was not present in unrelated elephants. This mutation causes an amino acid substitution (p.Arg68Gly) that is predicted to be deleterious. Two living offspring of the affected elephant were heterozygous for the mutation and had normal plasma FVII activities and coagulation profiles. Tissue from a third offspring, a deceased calf, was utilized to show that it was also a heterozygote. A DNA test has been developed to enable the screening of additional elephants for this mutation. Consistent with FVII deficiency investigations in other species, the condition did not cause a serious bleeding tendency in this individual elephant.

  12. Characterization of carbonic anhydrase XIII in the erythrocytes of the Burmese python, Python molurus bivittatus.

    Science.gov (United States)

    Esbaugh, A J; Secor, S M; Grosell, M

    2015-09-01

    Carbonic anhydrase (CA) is one of the most abundant proteins found in vertebrate erythrocytes with the majority of species expressing a low activity CA I and high activity CA II. However, several phylogenetic gaps remain in our understanding of the expansion of cytoplasmic CA in vertebrate erythrocytes. In particular, very little is known about isoforms from reptiles. The current study sought to characterize the erythrocyte isoforms from two squamate species, Python molurus and Nerodia rhombifer, which was combined with information from recent genome projects to address this important phylogenetic gap. Obtained sequences grouped closely with CA XIII in phylogenetic analyses. CA II mRNA transcripts were also found in erythrocytes, but found at less than half the levels of CA XIII. Structural analysis suggested similar biochemical activity as the respective mammalian isoforms, with CA XIII being a low activity isoform. Biochemical characterization verified that the majority of CA activity in the erythrocytes was due to a high activity CA II-like isoform; however, titration with copper supported the presence of two CA pools. The CA II-like pool accounted for 90 % of the total activity. To assess potential disparate roles of these isoforms a feeding stress was used to up-regulate CO2 excretion pathways. Significant up-regulation of CA II and the anion exchanger was observed; CA XIII was strongly down-regulated. While these results do not provide insight into the role of CA XIII in the erythrocytes, they do suggest that the presence of two isoforms is not simply a case of physiological redundancy.

  13. Screening a coastal population in Southern Italy: iodine deficiency and prevalence of goitre, nutritional aspects and cardiovascular risk factors.

    Science.gov (United States)

    Valentino, R; Savastano, S; Tommaselli, A P; Di Biase, S; Calvanese, E; Carbone, D; Dorato, M; Orio, F; Lupoli, G; Lombardi, G

    2004-02-01

    To evaluate the prevalence of goitre by means of urinary iodine excretion, palpatory and ultrasonographic thyroid examinations in a heterogeneous population living by the sea. We used a special self-administered questionnaire to evaluate thyroid size, iodine intake, eating habits and cardiovascular risk factors in 600 subjects with a mean age of 45 +/- 17 years: 253 men (42.3%) and 347 women (57.7%). Urinary iodine excretion was low (72.1 +/- 15.7 microg/L; median 71.2) and associated with ultrasonographic evidence of an enlarged thyroid (16%) or structural thyroid abnormalities (30%), thus allowing us to define the Salerno Gulf as a mild-moderate area of endemic goitre. All of the subjects ate a Mediterranean diet, with a mean of two portions of fish/week. The cardiovascular risk factors considered were obesity, cigarette smoking, hypertension, hypercholesterolemia, hypertriglyceridemia and diabetes, the prevalences of which were in line with those reported in other studies of similar age-matched populations. The moderate intake of fish and the consumption of a Mediterranean diet did not prevent goitre. Iodine deficiency and subsequent goitre endemia are also present at sea level, probably because of a diet based on local products grown on soil with a low iodine content or possible seawater, soil and air environmental pollution that may interfere with the availability of iodine. The assessment of iodine deficiency should therefore involve the entire population and not only subjects living far from the sea.

  14. Cobalamin deficiency.

    Science.gov (United States)

    Herrmann, Wolfgang; Obeid, Rima

    2012-01-01

    Cobalamin (Cbl, vitamin B12) consists of a corrinoid structure with cobalt in the centre of the molecule. Neither humans nor animals are able to synthesize this vitamin. Foods of animal source are the only natural source of cobalamin in human diet. There are only two enzymatic reactions in mammalian cells that require cobalamin as cofactor. Methylcobolamin is a cofactor for methionine synthase. The enzyme methylmalonyl-CoA-mutase requires adenosylcobalamin as a cofactor. Therefore, serum concentrations of homocysteine (tHcy) and methylmalonic acid (MMA) will increase in cobalamin deficiency. The cobalamin absorption from diet is a complex process that involves different proteins: haptocorrin, intrinsic factor and transcobalamin (TC). Cobalamin that is bound to TC is called holotranscobalamin (holoTC) which is the metabolically active vitamin B12 fraction. HoloTC consists 6 and 20% of total cobalamin whereas 80% of total serum cobalamin is bound to another binding protein, haptocorrin. Cobalamin deficiency is common worldwide. Cobalamin malabsorption is common in elderly subjects which might explain low vitamin status. Subjects who ingest low amount of cobalamin like vegetarians develop vitamin deficiency. No single parameter can be used to diagnose cobalamin deficiency. Total serum cobalamin is neither sensitive nor it is specific for cobalamin deficiency. This might explain why many deficient subjects would be overlooked by utilizing total cobalamin as status marker. Concentration of holotranscobalamin (holoTC) in serum is an earlier marker that becomes decreased before total serum cobalamin. Concentrations of MMA and tHcy increase in blood of cobalamin deficient subjects. Despite limitations of these markers in patients with renal dysfunction, concentrations of MMA and tHcy are useful functional markers of cobalamin status. The combined use of holoTC and MMA assays may better indicate cobalamin status than either of them. Because Cbl deficiency is a risk factor

  15. Impaired secretion of carboxyl-terminal truncated factor VII due to an F7 nonsense mutation associated with FVII deficiency.

    Science.gov (United States)

    Tanaka, Ryoko; Nakashima, Daisuke; Suzuki, Atsuo; Miyawaki, Yuhri; Fujimori, Yuta; Yamada, Takayuki; Takagi, Akira; Murate, Takashi; Yamamoto, Koji; Katsumi, Akira; Matsushita, Tadashi; Naoe, Tomoki; Kojima, Tetsuhito

    2010-03-01

    Factor VII (FVII) is a vitamin K-dependent glycoprotein secreted into the blood circulation from hepatic cells. We investigated the molecular basis of the congenital FVII deficiency found in a Japanese patient. We analyzed the F7 gene of the patient, who was diagnosed with a FVII deficiency at pregnancy. We expressed a carboxyl-terminal truncated FVII (Arg462X FVII) corresponding to the identified mutation in CHO-K1 cells. To study roles of the carboxyl-terminus in the secretion of FVII, we also expressed a series of recombinant FVIIs deleted of limited numbers of carboxyl-terminal amino acids (462Arg-466Pro). We identified a nonsense mutation (c.1384C>T: p.Arg462X) in F7, leading to a lack of five amino acids in the carboxyl-terminus. In expression experiments, Arg462X FVII was undetectable not only by Western blotting, but also by ELISA. A Western blot analysis of the truncated FVIIs revealed that all mutants were expressed in the cells the same as the wild type, but were secreted into the culture medium in lesser amounts than the wild type depending on the length of the deletion, which was confirmed by ELISA. Arg462X FVII did not colocalize with the Golgi on immunofluorescence staining, suggesting that it might be retained in the ER and degraded in the cell. The carboxyl-terminal amino acids of FVII play an important role in its secretion, and the p.Arg462X mutation was likely to have caused the FVII deficiency in this patient. (c) 2009 Elsevier Ltd. All rights reserved.

  16. Identification of Factor XI Deficiency in Dairy Cattle%中国荷斯坦牛凝血因子XI缺陷症遗传分析

    Institute of Scientific and Technical Information of China (English)

    东天; 谢岩; 孙东晓; 张沅; 张胜利; 张毅; 刘林; 陈绍祜

    2011-01-01

    Factor XI deficiency is an autosomal recessive inherited defect in Holstein cattle. Affected animals showed a number of symptoms adversely affecting the reproductive performances of the cows and appeared more susceptible to diseases. The mechanism on this deficiency is the mutation for a 76 base insertion into exon 12 of FXI gene that causes Factor XI deficiency. In this study, based on the PCR method, 571 bulls from 13 bull stations were detected for the genotypes of Factor XI deficiency. No carrier bulls of Factor XI deficiency were found.%凝血因子XI缺陷症(FactorXIdenciency)是荷斯坦牛的一种常染色体单基因控制的隐性遗传缺陷。该病的遗传基础是由位于牛第27号染色体的凝血因子XI基因外显子12上发生的一段76bp序列插入。本研究采用PCR方法对我国13个主要公牛站的571头荷斯坦公牛的凝血因子XI基因进行了全面检测,未发现隐性有害基因携带者和纯合个体。

  17. Current prevalence of goiter determined by ultrasonography and associated risk factors in a formerly iodine-deficient area of Turkey.

    Science.gov (United States)

    Kocak, Mustafa; Erem, Cihangir; Deger, Orhan; Topbas, Murat; Ersoz, Halil Onder; Can, Emine

    2014-09-01

    The aim of this study was to determine the prevalence of goiter and related risk factors in an adult population in a formerly iodine-deficient area of Turkey. In this cross-sectional study, we enrolled 2,500 subjects (1,270 women and 1,230 men, aged over 20 years) by multistage sampling. Blood and urine specimens were collected for the assessment of thyroid function. Thyroid ultrasonography (USG) was performed to measure thyroid volume and evaluate nodules. The overall goiter prevalence was 26.5 % (28.4 % in women, 24.5 % in men, P iodine was 122.79 μg/L. USG revealed thyroid nodules in 35.2 % of the subjects (38.4 % in women, 31.8 % in men, P iodine excretion levels, prevalence of goiter in this adult population in a formerly iodine-deficient province of Turkey remained high, even about 10 years after salt iodine supplementation program introduction. In addition, the goiter prevalence was higher for female gender, advanced age, positive family history of goiter, low education level, and high BMI.

  18. Effects of iron deficiency anemia and its treatment on fibroblast growth factor 23 and phosphate homeostasis in women.

    Science.gov (United States)

    Wolf, Myles; Koch, Todd A; Bregman, David B

    2013-08-01

    Fibroblast growth factor 23 (FGF23) is an osteocyte-derived hormone that regulates phosphate and vitamin D homeostasis. Through unknown mechanisms, certain intravenous iron preparations induce acute, reversible increases in circulating FGF23 levels that lower serum phosphate in association with inappropriately low levels of calcitriol, similar to genetic diseases of primary FGF23 excess. In contrast, studies in wild-type mice suggest that iron deficiency stimulates fgf23 transcription but does not result in hypophosphatemia because FGF23 is cleaved within osteocytes by an unknown catabolic system. We tested the association of iron deficiency anemia with C-terminal FGF23 (cFGF23) and intact FGF23 (iFGF23) levels in 55 women with a history of heavy uterine bleeding, and assessed the longitudinal biochemical response over 35 days to equivalent doses of randomly-assigned, intravenous elemental iron in the form of ferric carboxymaltose (FCM) or iron dextran. Iron deficiency was associated with markedly elevated cFGF23 (807.8 ± 123.9 relative units [RU]/mL) but normal iFGF23 (28.5 ± 1.1 pg/mL) levels at baseline. Within 24 hours of iron administration, cFGF23 levels fell by approximately 80% in both groups. In contrast, iFGF23 transiently increased in the FCM group alone, and was followed by a transient, asymptomatic reduction in serum phosphate iron dextran group. Reduced serum phosphate was accompanied by increased urinary fractional excretion of phosphate, decreased calcitriol levels, and increased parathyroid hormone levels. These findings suggest that iron deficiency increases cFGF23 levels, and that certain iron preparations temporarily increase iFGF23 levels. We propose that intravenous iron lowers cFGF23 in humans by reducing fgf23 transcription as it does in mice, whereas carbohydrate moieties in certain iron preparations may simultaneously inhibit FGF23 degradation in osteocytes leading to transient increases in iFGF23 and reduced serum phosphate.

  19. Aerobic Exercise Suppresses Atherosclerosis Through Adiponectin-Nuclear Transcription Factor κB Pathway in Apolipoprotein E-deficient Mice.

    Science.gov (United States)

    Wu, Weidong; Wang, Haiying; Jiao, Guangfa; Yue, Jingjing; Wang, Guowei

    2017-03-01

    We aimed to investigate the therapeutic effect of aerobic exercise on atherosclerosis (AS) in apolipoprotein E-deficient mice and the adiponectin (ApN)-nuclear transcription factor κB (NF-κB) pathway involved in the related anti-inflammation. ApoE-deficient mice with AS (AS+C), and ApoE-deficient mice with AS and aerobic exercise (AS+E) were investigated for body weight and visceral fat. Pathomorphology of the aortic vascular wall was qualitatively and quantitatively evaluated with hematoxylin-eosin staining. The ApN messenger RNA level in adipose tissue and ApN level in plasma were determined. The aortic adiponectin receptor 1 (AdipoR1) and NF-κB levels were determined with western blot. There was no significant difference in body weight between the AS+C and the AS+E groups, but visceral fat in the AS+E group was significantly smaller than that in the AS+C group. Aortic vascular wall fiber board in the AS+C group broke, but this aortic disease in the AS+E group was obviously alleviated. The AS+E group showed a smaller neointimal hyperplasia and plaque area compared with AS+C group. After a high-fat diet, the ApN levels in both adipose tissue and plasma were decreased in the AS+C group and returned to a relative high level in the AS+E group. The expression of AdipoR1 protein in the AS+C group was significantly lower than those in the AS+E group. As for NF-κB protein, its enhanced expression in the AS+C group was reversed to a relatively low level in the AS+E group. Aerobic exercise suppressed AS through the ApN-NF-κB pathway in ApoE-deficient mice. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  20. Autism as a disorder of deficiency of brain-derived neurotrophic factor and altered metabolism of polyunsaturated fatty acids.

    Science.gov (United States)

    Das, Undurti N

    2013-10-01

    Autism has a strong genetic and environmental basis in which inflammatory markers and factors concerned with synapse formation, nerve transmission, and information processing such as brain-derived neurotrophic factor (BDNF), polyunsaturated fatty acids (PUFAs): arachidonic (AA), eicosapentaenoic (EPA), and docosahexaenoic acids (DHA) and their products and neurotransmitters: dopamine, serotonin, acetylcholine, γ-aminobutyric acid, and catecholamines and cytokines are altered. Antioxidants, vitamins, minerals, and trace elements are needed for the normal metabolism of neurotrophic factors, eicosanoids, and neurotransmitters, supporting reports of their alterations in autism. But, the exact relationship among these factors and their interaction with genes and proteins concerned with brain development and growth is not clear. It is suggested that maternal infections and inflammation and adverse events during intrauterine growth of the fetus could lead to alterations in the gene expression profile and proteomics that results in dysfunction of the neuronal function and neurotransmitters, alteration(s) in the metabolism of PUFAs and their metabolites resulting in excess production of proinflammatory eicosanoids and cytokines and a deficiency of anti-inflammatory cytokines and bioactive lipids that ultimately results in the development of autism. Based on these evidences, it is proposed that selective delivery of BDNF and methods designed to augment the production of anti-inflammatory cytokines and eicosanoids and PUFAs may prevent, arrest, or reverse the autism disease process.

  1. Prevalence and risk factors of micronutrient deficiencies pre- and post-antiretroviral therapy (ART) among a diverse multicountry cohort of HIV-infected adults.

    Science.gov (United States)

    Shivakoti, Rupak; Christian, Parul; Yang, Wei-Teng; Gupte, Nikhil; Mwelase, Noluthando; Kanyama, Cecilia; Pillay, Sandy; Samaneka, Wadzanai; Santos, Breno; Poongulali, Selvamuthu; Tripathy, Srikanth; Riviere, Cynthia; Berendes, Sima; Lama, Javier R; Cardoso, Sandra W; Sugandhavesa, Patcharaphan; Tang, Alice M; Semba, Richard D; Campbell, Thomas B; Gupta, Amita

    2016-02-01

    HIV-infected adults have increased risk of several individual micronutrient deficiencies. However, the prevalence and risk factors of concurrent and multiple micronutrient deficiencies and whether micronutrient concentrations change after antiretroviral therapy (ART) initiation have not been well described. The objective of this study was to determine the prevalence and risk factors of individual, concurrent and multiple micronutrient deficiencies among ART-naïve HIV-infected adults from nine countries and assess change in micronutrient status 48 weeks post-ART initiation. A random sub-cohort (n = 270) stratified by country was selected from the multinational PEARLS clinical trial (n = 1571 ART-naïve, HIV-infected adults). We measured serum concentrations of vitamins A, D (25-hydroxyvitamin), E, carotenoids and selenium pre-ART and 48 weeks post-ART initiation, and measured vitamins B6, B12, ferritin and soluble transferrin receptor at baseline only. Prevalence of single micronutrient deficiencies, concurrent (2 coexisting) or conditional (a deficiency in one micronutrient given a deficiency in another) and multiple (≥3) were determined using defined serum concentration cutoffs. We assessed mean changes in micronutrient concentrations from pre-ART to week 48 post-ART initiation using multivariable random effects models. Of 270 participants, 13.9%, 29.2%, 24.5% and 32.4% had 0, 1, 2 and multiple deficiencies, respectively. Pre-ART prevalence was the highest for single deficiencies of selenium (53.2%), vitamin D (42.4%), and B6 (37.3%) with 12.1% having concurrent deficiencies of all three micronutrients. Deficiency prevalence varied widely by country. 48 weeks post-ART initiation, mean vitamin A concentration increased (p ART (p ART initiation but vary between countries. Importantly, despite increases in micronutrient concentrations, prevalence of individual deficiencies remains largely unchanged after 48 weeks on ART. Our results suggest that ART alone is

  2. Roux-en-Y gastric bypass surgery is effective in fibroblast growth factor-21 deficient mice

    Directory of Open Access Journals (Sweden)

    Christopher D. Morrison

    2016-10-01

    Conclusions: FGF21 signaling is not a critical single factor for the beneficial metabolic effects of RYGB. This may open up the possibility to use FGF21 as adjuvant therapy in patients with ineffective bariatric surgeries.

  3. Deficiency of Growth Differentiation Factor 3 Protects against Diet-Induced Obesity by Selectively Acting on White Adipose

    Science.gov (United States)

    Shen, Joseph J.; Huang, Lihua; Li, Liunan; Jorgez, Carolina; Matzuk, Martin M.; Brown, Chester W.

    2009-01-01

    Growth differentiation factor 3 (GDF3) is a member of the TGFβ superfamily. White adipose is one of the tissues in which Gdf3 is expressed, and it is the only tissue in which expression increases in response to high-fat diet. We generated Gdf3−/− mice, which were indistinguishable from wild-type mice and had normal weight curves on regular diet. However, on high-fat diet Gdf3−/− mice were resistant to the obesity that normally develops in wild-type mice. Herein we investigate the physiological and molecular mechanisms that underlie this protection from diet-induced obesity and demonstrate that GDF3 deficiency selectively affects white adipose through its influence on basal metabolic rates. Our results are consistent with a role for GDF3 in adipose tissue, with consequential effects on energy expenditure that ultimately impact adiposity. PMID:19008465

  4. Evaluation of the effect of triterpenes on urinary risk factors of stone formation in pyridoxine deficient hyperoxaluric rats.

    Science.gov (United States)

    Vidya, Lakshminarasimhan; Lenin, Mahimainathan; Varalakshmi, Palaninathan

    2002-09-01

    Investigations were carried out to evaluate the efficacy of the pentacyclic triterpene, lupeol and its ester, lupeol linoleate, against calcium oxalate urolithiasis in rats. Administration of a pyridoxine deficient diet containing 3% glycollic acid for 21 days led to increased excretion of stone forming constituents such as calcium, oxalate and uric acid. Crystal deposition and subsequent renal tubular damage resulted in increased excretion of the tubular enzymes alkaline phosphatase, lactate dehydrogenase, gamma glutamyl transferase, beta glucuronidase and N-acetyl glucosaminidase along with reduced fibrinolytic enzymes. A reduction in the urinary inhibitory factors magnesium and glycosaminoglycans was also observed. Treatment with lupeol and lupeol linoleate reduced the extent of tubular damage as evidenced from reduced enzymuria and minimized the excretion of stone forming constituents.

  5. Tissue-type plasminogen activator deficiency delays bone repair: roles of osteoblastic proliferation and vascular endothelial growth factor.

    Science.gov (United States)

    Kawao, Naoyuki; Tamura, Yukinori; Okumoto, Katsumi; Yano, Masato; Okada, Kiyotaka; Matsuo, Osamu; Kaji, Hiroshi

    2014-08-01

    Further development in research of bone regeneration is necessary to meet the clinical demand for bone reconstruction. Recently, we reported that plasminogen is crucial for bone repair through enhancement of vessel formation. However, the details of the role of tissue-type plasminogen activator (tPA) and urokinase-type plasminogen activator (uPA) in the bone repair process still remain unknown. Herein, we examined the effects of plasminogen activators on bone repair after a femoral bone defect using tPA-deficient (tPA(-/-)) and uPA-deficient (uPA(-/-)) mice. Bone repair of the femur was delayed in tPA(-/-) mice, unlike that in wild-type (tPA(+/+)) mice. Conversely, the bone repair was comparable between wild-type (uPA(+/+)) and uPA(-/-) mice. The number of proliferative osteoblasts was decreased at the site of bone damage in tPA(-/-) mice. Moreover, the proliferation of primary calvarial osteoblasts was reduced in tPA(-/-) mice. Recombinant tPA facilitated the proliferation of mouse osteoblastic MC3T3-E1 cells. The proliferation enhanced by tPA was antagonized by the inhibition of endogenous annexin 2 by siRNA and by the inhibition of extracellular signal-regulated kinase (ERK)1/2 phosphorylation in MC3T3-E1 cells. Vessel formation as well as the levels of vascular endothelial growth factor (VEGF) and hypoxia-inducible factor-1α (HIF-1α) were decreased at the damaged site in tPA(-/-) mice. Our results provide novel evidence that tPA is crucial for bone repair through the facilitation of osteoblast proliferation related to annexin 2 and ERK1/2 as well as enhancement of vessel formation related to VEGF and HIF-1α at the site of bone damage. Copyright © 2014 the American Physiological Society.

  6. Membrane Omega-3 Fatty Acid Deficiency as a Preventable Risk Factor for Comorbid Coronary Heart Disease in Major Depressive Disorder

    Directory of Open Access Journals (Sweden)

    Robert K. McNamara

    2009-01-01

    Full Text Available Major depression disorder (MDD significantly increases the risk for coronary heart disease (CHD which is a leading cause of mortality in patients with MDD. Moreover, depression is frequently observed in a subset of patients following acute coronary syndrome (ACS and increases risk for mortality. Here evidence implicating omega-3 (n-3 fatty acid deficiency in the pathoaetiology of CHD and MDD is reviewed, and the hypothesis that n-3 fatty acid deficiency is a preventable risk factor for CHD comorbidity in MDD patients is evaluated. This hypothesis is supported by cross-national and cross-sectional epidemiological surveys finding an inverse correlation between n-3 fatty acid status and prevalence rates of both CHD and MDD, prospective studies finding that lower dietary or membrane EPA+DHA levels increase risk for both MDD and CHD, case-control studies finding that the n-3 fatty acid status of MDD patients places them at high risk for emergent CHD morbidity and mortality, meta-analyses of controlled n-3 fatty acid intervention studies finding significant advantage over placebo for reducing depression symptom severity in MDD patients, and for secondary prevention of cardiac events in CHD patients, findings that n-3 fatty acid status is inversely correlated with other documented CHD risk factors, and patients diagnosed with MDD after ACS exhibit significantly lower n-3 fatty acid status compared with nondepressed ACS patients. This body of evidence provides strong support for future studies to evaluate the effects of increasing dietary n-3 fatty acid status on CHD comorbidity and mortality in MDD patients.

  7. Increased depression-like behaviors in corticotropin-releasing factor receptor-2-deficient mice: sexually dichotomous responses.

    Science.gov (United States)

    Bale, Tracy L; Vale, Wylie W

    2003-06-15

    Depressive disorders affect nearly 19 million American adults, making depression and the susceptibility for developing depression a critical focus of mental health research today. Females are twice as likely to develop depression as males. Stress is a known risk factor for developing depression, and recent hypotheses suggest an involvement of an overactive stress axis. As mediators of the stress response, corticotropin-releasing factor (CRF) and its receptors (CRFR1 and CRFR2) have been implicated in the propensity for developing stress-related mood disorders. Mice deficient in CRFR2 display increased anxiety-like behaviors and a hypersensitive stress response. As a possible animal model of depression, these mice were tested for depression-like behaviors in the forced swim test. Comparisons were made between wild-type and mutant animals, as well as between sexes. Male and female CRFR2-mutant mice showed increased immobility as an indicator of depression compared with wild-type mice of the same sex. In addition, mutant and wild-type female mice demonstrated increased immobile time compared with males of the same genotype. Treatment of CRFR2-deficient mice with the CRFR1 antagonist antalarmin decreased immobile time and increased swim time in both sexes. We found a significant effect of sex for both time spent immobile and swimming after antalarmin treatment. Because differences in behaviors in the forced swim test are good indicators of serotonergic and catecholaminergic involvement, our results may reveal an interaction of CRF pathways with other known antidepressant systems and may also support an involvement of CRF receptors in the development of depression such that elevated CRFR1 activity, in the absence of CRFR2, increases depression-like behaviors.

  8. Dirac R-matrix calculations of photoionization cross-sections of Ni XIII

    Science.gov (United States)

    Sardar, S.; Bilal, M.; Bari, M. A.; Nazir, R. T.; Hannan, A.; Salahuddin, M.; Nasim, M. H.

    2016-05-01

    In this paper, we report total photoionization cross-sections of Ni XIII in the ground state (3P2) and four excited states (3P1,0, 1D2, 1S0) for the first time over the photon energy range 380-480 eV. The target wavefunctions are constructed with fully relativistic atomic structure GRASP code. Our calculated energy levels and oscillator strengths of core ion Ni XIV agree well with available experimental and theoretical results. The ionization threshold value of ground state of Ni XIII is found to be more closer to the experimental ionization energy and improved over the previous calculations. The photoionization cross-sections are calculated using the fully relativistic DARC code with an appropriate energy step of 0.01 eV to delineate the resonance structures. The calculated ionization cross-sections are important for the modelling of features of photoionized plasmas and for stellar opacities.

  9. In Vivo Gene Therapy of Hemophilia B: Sustained Partial Correction in Factor IX-Deficient Dogs

    Science.gov (United States)

    Kay, Mark A.; Rothenberg, Steven; Landen, Charles N.; Bellinger, Dwight A.; Leland, Frances; Toman, Carol; Finegold, Milton; Thompson, Arthur R.; Read, M. S.; Brinkhous, Kenneth M.; Woo, Savio L. C.

    1993-10-01

    The liver represents a model organ for gene therapy. A method has been developed for hepatic gene transfer in vivo by the direct infusion of recombinant retroviral vectors into the portal vasculature, which results in the persistent expression of exogenous genes. To determine if these technologies are applicable for the treatment of hemophilia B patients, preclinical efficacy studies were done in a hemophilia B dog model. When the canine factor IX complementary DNA was transduced directly into the hepatocytes of affected dogs in vivo, the animals constitutively expressed low levels of canine factor IX for more than 5 months. Persistent expression of the clotting. factor resulted in reductions of whole blood clotting and partial thromboplastin times of the treated animals. Thus, long-term treatment of hemophilia B patients may be feasible by direct hepatic gene therapy in vivo.

  10. Factors of Innate and Adaptive Local Immunity in Children with Primary Deficiencies of Antibody Formation

    Directory of Open Access Journals (Sweden)

    L.I. Chernyshova

    2013-11-01

    Full Text Available In 40 children with various types of primary immunodeficiencies (PID of antibody formation we examined factors of local immunity in saliva. It is found that in the saliva of children with PID of antibody formation in comparison with immunocompetent children the concentration of factors of adaptive immunity is significantly reduced. Lack of adaptive immunity in the PID of antibody formation to some extent is compensated by increased concentrations of innate immune factors on the mucous membranes — the free Sc, as well as lactoferrin in selective immunodeficiency of IgA. At PID of antibody formation we observed increased TNF-α level in the saliva, which may indicate the persistence of local inflammation on the membranes of the respiratory tract.

  11. Factors contributing to the biofilm-deficient phenotype of Staphylococcus aureus sarA mutants.

    Directory of Open Access Journals (Sweden)

    Laura H Tsang

    demonstrate that the inability of a sarA mutant to repress production of extracellular nuclease and multiple proteases have independent but cumulative effects that make a significant contribution to the biofilm-deficient phenotype of an S. aureus sarA mutant.

  12. Diagnostic difficulties of factor XI deficiencies: interferences' assay or real deficit?

    Science.gov (United States)

    Gaymard, Alexandre; Nougier, Christophe

    2016-06-01

    Madam P, 77 years old, consulted in the hemostasis department after a coagulation anomaly was discovered during her preoperative test for a total hip prosthesis. After confirmation of a persistent and increased aPTT, additional tests were performed and showed the presence of antiphospholipid antibodies. Factor VIII level could be corrected after the plasma dilution to 1/40(th). But successive dilutions were not enough to obtain a correct factor IX (FIX) and factor XI (FXI) level. FIX level was obtained by chromogenic method in order to avoid the interferences caused by the antibodies. Finally, despite the change of reagents and dilutions up to 1/160(th), the FXI level couldn't be determined. Despite these results and those of the thrombin generation assay, the surgery was successfully done without specific treatment thanks to the absence of hemorrhagic history. This observation highlights the diagnostic and monitoring difficulties for uncommon clotting factor deficit. The development of interference free test could increase the support for these patients.

  13. Evidence of independent evolution of genotype XIII Newcastle disease viruses in India.

    Science.gov (United States)

    Das, Moushumee; Kumar, Sachin

    2017-04-01

    Despite the prevalence of Newcastle disease virus (NDV) outbreaks in India through the decades, there has been little genetic characterisation of the virulent strains circulating in Northeast India. In 2014, a poultry farm in the Kamrup district of Assam reported an ND outbreak. In this study, genetic analysis and clinicopathological tests showed the virulent nature of the isolate Kamrup. Based on prudent classification criteria, the virulent strain Kamrup was found to be most closely related to members of genotype XIII of class II NDV. A phylogenetic analysis of NDV strains suggested three sub-genotypes: XIIIa, XIIIb and XIIIc. NDV strain Kamrup belonged to sub-genotype XIIIc. Sub-genotype XIIIc isolates were similar to the 1982 isolate from cockatoo and appeared to have evolved parallel to the preceding genotype XIII viruses circulating in India. The high genetic diversity and frequency of mutations observed in the envelope glycoproteins of strain Kamrup demonstrate the evolution of the pandemic genotype XIII NDV in India, which further undermines and complicates of NDV management in India.

  14. Experimental and clinical evidence of the role of cytokines and growth factors in the pathogenesis of acquired cobalamin-deficient leukoneuropathy.

    Science.gov (United States)

    Scalabrino, Giuseppe; Veber, Daniela; Mutti, Elena

    2008-11-01

    Our experimental and clinical studies have highlighted the non-coenzyme functions of cobalamin (Cbl; vitamin B12). The neuropathy of the rat central nervous system (CNS) due to Cbl deficiency is associated with increases in CNS tissue and/or cerebrospinal fluid (CSF) levels of some neurotoxic molecules, and decreases in local and/or CSF levels of some neurotrophic molecules. The increased molecules are nerve growth factor (NGF), tumor necrosis factor (TNF)-alpha, and the soluble (s)CD40:sCD40 Ligand dyad; the decreased molecules are epidermal growth factor (EGF) and interleukin-6. The morphological lesions of the CNS white matter in Cbl-deficient (Cbl-Df) rats can be prevented to the same extent by treatments replacing Cbl or the deficient neurotrophic molecules, or treatment with agents that antagonize the excess neurotoxic molecules. Patients with neurological and/or hematological manifestations of severe Cbl deficiency also have high TNF-alpha levels and low EGF levels in CSF and serum. Cbl replacement treatment corrects cytokine and growth factor abnormalities in Cbl-Df patients and Cbl-Df rats, and so Cbl-Df CNS neuropathy is also due to an imbalance in local cytokine/growth factor networks. TNF-alpha and NGF levels are also increased in Cbl-Df rat liver, which is morphologically unaffected by Cbl deficiency. The increases in TNF-alpha and NGF levels increase nuclear factor-kappaB activity levels in both the CNS and liver, and this indirect regulation supports the idea that Cbl may modulate the expression of some cytokine/growth factor genes in rat CNS and other tissues. Finally, we have tried to harmonize our pathogenetic theory of cytokine and growth factor dysregulation with the biochemical interpretation.

  15. related Factors of chronic Energy Deficiency at Pregnant Woman in kamoning and Tambelangan Sub District, Sampang District, West Java

    Directory of Open Access Journals (Sweden)

    Vita Kartika Mahirawati

    2015-01-01

    Full Text Available background:Pregnant woman who suffered Chronic Energy Deficiency (CED has delivered babies with low birth weight and increased the risk for mortality. The objective of this research was to analyze the related factors of CED at pregnant woman. Method:A Cross sectional design was used. A random sample of pregnant women who registered in kohort. has taken 104 people as the samples. Collecting data using questionnaires (quantitative and secondary data obtain through kohort register during 2012–2013 and profile of Dinkes 2012, conducted during 6 (six months 2013 in Puskesmas Kamoning and Tambelangan. The data were analyzed descriptively and statistically using Chi Square test. result: The socio-economic factors: 35.5% mother’s education mostly elementary school graduates. 29.4 % didn’t graduate from elementary school. The majority of their husband (39.2% work as farm worker with income less than 1 million rupiah per month. The maternal factors: the average age between 21 until 34 years old (70.6%. The age of marriage mostly under 17 years old (41.1% and most of them (31.4% have their first pregnancy in the age between 18 until 21 years old. The most pregnant women (54.9% ate 3 times/day. 70.6% pregnant women with CED suffered from anemia; 66.7% of them took irow tablets every day. conclusion:economic factors social conditions associated with the incidence of maternal CED husband is education and maternal work status, whereas maternal factors are gestational age, hemoglobin levels in the blood and consumption of iron pills. recommendation:Areas programme and sectorce, should be involved to reduce the CED prevalence.

  16. Evaluation of failure or deficient performance factors of approved research projects by TUMS research council

    Directory of Open Access Journals (Sweden)

    Zarinara A.

    2002-08-01

    Full Text Available The major activity for vice chancellor for research at Tehran University of Medical Sciences (TUMS is to monitor research projects undertaken by the academic staff. Sometimes projects have been failed in meeting their objectives. The aim of this project was the evaluation of the failure of the research projects at TUMS from the establishment date to 1379. A case-control survey was designed and 70 failed projects with 71 successfully completed projects were considered and compared. Results showed that type of study, location of administration, methodology, qualification of executive and duration of project were the factors of failure of the projects. Number of executives, experienced in research projects and executive position were not the effective factors. The most important reason for failure was unsustainably of executive. So, university research council (URC should pay attention on forecast of time and budget of projects and realization of mentioned matters, besides of attention to methodology of project.

  17. Disruption of the suprachiasmatic nucleus in fibroblast growth factor signaling-deficient mice

    OpenAIRE

    Ann Virginia Miller; Scott eKavanaugh; Pei-San eTsai

    2016-01-01

    Fibroblast growth factor (Fgf) 8 is essential for the development of multiple brain regions. Previous studies from our laboratory showed that reduced Fgf8 signaling led to the developmental alterations of neuroendocrine nuclei that originated within the diencephalon, including the paraventricular (PVN) and supraoptic (SON) nuclei. To further understand the role of Fgf8 in the development of other hypothalamic nuclei, we examined if Fgf8 and its cognate receptor, Fgfr1, also impact the integ...

  18. Disruption of the Suprachiasmatic Nucleus in Fibroblast Growth Factor Signaling-Deficient Mice

    OpenAIRE

    Miller, Ann V.; Kavanaugh, Scott I.; Tsai, Pei-San

    2016-01-01

    Fibroblast growth factor (Fgf) 8 is essential for the development of multiple brain regions. Previous studies from our laboratory showed that reduced Fgf8 signaling led to the developmental alterations of neuroendocrine nuclei that originated within the diencephalon, including the paraventricular (PVN) and supraoptic (SON) nuclei. To further understand the role of Fgf8 in the development of other hypothalamic nuclei, we examined if Fgf8 and its cognate receptor, Fgfr1, also impact the integri...

  19. A severe deficiency of coagulation factor VIIa results in attenuation of the asthmatic response in mice.

    Science.gov (United States)

    Shinagawa, Kazuhiko; Ploplis, Victoria A; Castellino, Francis J

    2009-05-01

    Eosinophil counts in the bronchoalveolar lavage fluid of wild-type (WT) mice increased after ovalbumin (OVA) challenge, a response that was diminished in comparably challenged low-expressing coagulation factor VII (FVII(tTA/tTA)) mice. Levels of T helper type 2 (Th2) cytokines, IL-4, IL-5, and IL-13, and eosinophil-attracting chemokines, eotaxin and RANTES, were also lower in the OVA-challenged FVII(tTA/tTA) mice. Eosinophils purified from low-FVII mice underwent apoptosis at a faster rate compared with WT eosinophils, and eosinophil migration in response to eotaxin was reduced in eosinophils obtained from FVII(tTA/tTA) mice. Airway hyperresponsiveness and mucous layer thickness were reduced in OVA-treated FVII(tTA/tTA) mice, and addition of exogenous coagulation factor X (FX) enhanced mucin production in human epithelial NCI-H292 cells. Correspondingly, incubation of FX with NCI-H292 cells resulted in activated (a) FX production, suggesting that the components required for FX activation were present on NCI-H292 cells. These results demonstrate that FVIIa functions in the asthmatic response to an allergen by stimulating lung eosinophilia, airway hyperresponsiveness, and mucin production, this latter effect through its ability to activate FX in conjunction with tissue factor.

  20. Vitamin D deficiency is a risk factor for obesity and diabetes type 2 in women at late reproductive age.

    Science.gov (United States)

    Grineva, E N; Karonova, T; Micheeva, E; Belyaeva, O; Nikitina, I L

    2013-07-01

    It was suggested that glucose metabolism and body fat content depend on serum levels of 25-hydroxyvitamin D [25(OH)D]. We studied 320 healthy women at late reproductive age of 40 to 52 years old (mean age 46.1±4.5) from St. Petersburg (North-West region of Russia). 25(ОН)D levels were from 19.4 to 134.0 nMol/L (mean 52.9±22.7). Vitamin D deficiency (lower than 50 nMol/L) and insufficiency (50-75 nMol/L) was revealed in 59.1% and 27.8% of women, respectively. The study showed that low 25(OH)D levels were associated with obesity (r=-0.35, p$#X003C0.01), increased plasma glucose levels after OGTT (r=-0.31, p$#X003C0.01) and decreased insulin sensitivity index (r=-0.28, p$#X003C0.01). We found that 25(OH)D levels below 50 nMol/L were associated with obesity risk (OR 2.25[1.05-3.95], CI 95%) but not with risk of impaired glucose metabolism (1.07[0.54-2.12],CI95%). Our results showed that vitamin D insufficiency is highly prevalent in the population of healthy women. Low 25(OH)D levels correlated with high body fat, glucose levels and decreased insulin sensitivity. We conclude that vitamin D deficiency is a potential risk factor for obesity and development of insulin resistance leading to diabetes type 2.

  1. Brain-derived neurotrophic factor deficiency restricts proliferation of oligodendrocyte progenitors following cuprizone-induced demyelination.

    Science.gov (United States)

    Tsiperson, Vladislav; Huang, Yangyang; Bagayogo, Issa; Song, Yeri; VonDran, Melissa W; DiCicco-Bloom, Emanuel; Dreyfus, Cheryl F

    2015-01-01

    Brain-derived neurotrophic factor (BDNF) is a member of the neurotrophin family of growth factors that through its neurotrophic tyrosine kinase, receptor, type 2 (TrkB) receptor, increases 5-bromo-2-deoxyuridine incorporation in oligodendrocyte progenitor cells (OPCs) in culture. Roles in vivo are less well understood; however, increases in numbers of OPCs are restricted in BDNF+/- mice following cuprizone-elicited demyelination. Here, we investigate whether these blunted increases in OPCs are associated with changes in proliferation. BDNF+/+ and BDNF+/- mice were fed cuprizone-containing or control feed. To assess effects on OPC numbers, platelet-derived growth factor receptor alpha (PDGFRα)+ or NG2+ cells were counted. To monitor DNA synthesis, 5-ethynyl-2'-deoxyuridine (EdU) was injected intraperitoneally and colocalized with PDGFRα+ cells. Alternatively, proliferating cell nuclear antigen (PCNA) was colocalized with PDGFRα or NG2. Labeling indices were determined in the BDNF+/+ and BDNF+/- animals. After 4 or 5 weeks of control feed, BDNF+/- mice exhibit similar numbers of OPCs compared with BDNF+/+ animals. The labeling indices for EdU and PCNA also were not significantly different, suggesting that neither the DNA synthesis phase (S phase) nor the proliferative pool size was different between genotypes. In contrast, when mice were challenged by cuprizone for 4 or 5 weeks, increases in OPCs observed in BDNF+/+ mice were reduced in the BDNF+/- mice. This difference in elevations in cell number was accompanied by decreases in EdU labeling and PCNA labeling without changes in cell death, indicating a reduction in the DNA synthesis and the proliferative pool. Therefore, levels of BDNF influence the proliferation of OPCs resulting from a demyelinating lesion.

  2. Determining The Factors Causing Human Error Deficiencies At A Public Utility Company

    Directory of Open Access Journals (Sweden)

    F. W. Badenhorst

    2004-11-01

    Full Text Available According to Neff (1977, as cited by Bergh (1995, the westernised culture considers work important for industrial mental health. Most individuals experience work positively, which creates a positive attitude. Should this positive attitude be inhibited, workers could lose concentration and become bored, potentially resulting in some form of human error. The aim of this research was to determine the factors responsible for human error events, which lead to power supply failures at Eskom power stations. Proposals were made for the reduction of these contributing factors towards improving plant performance. The target population was 700 panel operators in Eskom’s Power Generation Group. The results showed that factors leading to human error can be reduced or even eliminated. Opsomming Neff (1977 soos aangehaal deur Bergh (1995, skryf dat in die westerse kultuur werk belangrik vir bedryfsgeestesgesondheid is. Die meeste persone ervaar werk as positief, wat ’n positiewe gesindheid kweek. Indien hierdie positiewe gesindheid geïnhibeer word, kan dit lei tot ’n gebrek aan konsentrasie by die werkers. Werkers kan verveeld raak en dit kan weer lei tot menslike foute. Die doel van hierdie navorsing is om die faktore vas te stel wat tot menslike foute lei, en wat bydra tot onderbrekings in kragvoorsiening by Eskom kragstasies. Voorstelle is gemaak vir die vermindering van hierdie bydraende faktore ten einde die kragaanleg se prestasie te verbeter. Die teiken-populasie was 700 paneel-operateurs in die Kragopwekkingsgroep by Eskom. Die resultate dui daarop dat die faktore wat aanleiding gee tot menslike foute wel verminder, of geëlimineer kan word.

  3. The first cases of Fitzgerald factor deficiency in the Orient: three cases in one family.

    Science.gov (United States)

    Hayashi, H; Koya, H; Kuroda, M; Kitazima, K; Kimura, I; Katori, M; Oh-ishi, S

    1980-01-01

    Asymptomatic, female, 56-year-old identical Japanese twins were found to have a severe abnormality in the surface-mediated intrinsic coagulation, fibrinolysis and esterolytic activity. These defects were thought to be due to the lack of Fitzgerald factor, because of the prolongations of kaolin-activated partial thromboplastin time and kaolin-activated euglobulin lysis time that were not corrected by the addition of Fitzgerald trait-plasma but were corrected to normal levels by the addition of isolated bovine high-molecular-weight kininogen, Fletcher trait-plasma or Hageman trait-plasma.

  4. Melatonin-receptor-1-deficiency affects neurogenic differentiation factor immunoreaction in pancreatic islets and enteroendocrine cells of mice.

    Science.gov (United States)

    Shalabi, Andree; Fischer, Claudia; Korf, Horst-Werner; von Gall, Charlotte

    2013-09-01

    Neurogenic differentiation factor (NeuroD) is a transcription factor involved in the differentiation of neurons and in the control of energy balance and metabolism. It plays a key role in type 1 and type 2 diabetes. Melatonin is an important rhythmic endocrine signal within the circadian system of mammals and modulates insulin secretion and glucose metabolism. In the mouse pars tuberalis, NeuroD mRNA levels show day/night variation, which is independent of the molecular clock gene mPER1 but depends on the functional melatonin receptor 1 (MT1). So far, little is known about the effect of melatonin on NeuroD synthesis in the gastrointestinal tract. Thus, NeuroD protein levels and cellular localization were analyzed by immunohistochemistry in pancreatic islets and duodenal enteroendocrine cells of MT1- and mPER1-deficienct mice. In addition, the localization of NeuroD-positive cells was analyzed by double-immunofluorescence and confocal laser microscopy. In duodenal enteroendocrine cells and pancreatic islets of WT and PER1-deficient mice, NeuroD immunoreaction showed a peak during the early subjective night. In contrast, this peak was absent in MT1-deficent mice. These data suggest that melatonin, by acting on MT1 receptors, affects NeuroD expression in the gastrointestinal tract and thus might contribute to circadian regulation in metabolic functions.

  5. Proliferative response of fibroblasts expressing internalization-deficient epidermal growth factor (EGF) receptors is altered via differential EGF depletion effect.

    Science.gov (United States)

    Reddy, C C; Wells, A; Lauffenburger, D A

    1994-01-01

    We describe experiments comparing the proliferation responses to epidermal growth factor (EGF) by NR6 fibroblasts expressing genetically engineered epidermal growth factor receptors (EGFRs). These cells present either wild-type (WT) EGFR or a cytoplasmic domain-truncated (c'973) EGFR that exhibits a decreased ligand-induced internalization rate constant. In two distinct in vitro proliferation assays, with or without medium replenishment, we measured the specific cell proliferation rate constants and EGF depletion kinetics for both WT and c'973 cells. When EGF depletion is minimized by replenishment, the EGF concentration dependencies of the two cell types are similar, whereas when EGF depletion is not prevented, maximal proliferation of WT cells requires an initial EGF concentration that is approximately 10x that required by c'973 cells. However, when EGF depletion is accounted for, the dependencies of growth rate for the two cell types on the current EGF concentration in both assays are essentially identical. Our results demonstrate that diminished depletion of EGF from the extracellular medium is a major reason for increased mitogenic sensitivity to EGF by cells possessing internalization-deficient receptors.

  6. [Changes of acquired immune deficiency syndrome related knowledge, attitudes, behaviors and their influencing factors among college students in Beijing].

    Science.gov (United States)

    2017-06-18

    To compare acquired immune deficiency syndrome (AIDS) related knowledge, attitudes, behaviors and their influencing factors among college students in different years in Beijing, and to provide evidence for targeted health education among college students in future. College students were selected by the stratified cluster sampling method, and a questionnaire survey was conducted among college students in year 2006 and 2016 in Beijing. The sample sizes were 1 800 and 3 001 college students, respectively. The contents of the questionnaire included: socio-demographic characteristics, AIDS related knowledge, AIDS related attitude, sex intercourse and its related risk behaviors, condom use intension, and AIDS related health education. Compared with the year 2006, the average AIDS knowledge scores of college students in year 2016 dropped from 12.78±1.95 to 11.90±2.56 (t=12.91, Pinfluencing factors of intention on condom use were male (OR=0.713), self-efficacy of condom purchase (OR=0.876), never received sex education before college (OR=0.752), self-efficacy of condom use (OR=1.135), belief of condom use (OR=1.775), and attitude towards AIDS patients (OR=1.136). AIDS related knowledge, attitudes and behaviors among college students have been changed, AIDS related health education should be designed and improved based on new characteristics of college students. AIDS health education in colleges should pay more attention to sex attitude and sex responsibility and self-protection awareness among college students as well.

  7. A Comparative Study on Antioxidant System in Fish Hepatopancreas and Intestine Affected by Choline Deficiency: Different Change Patterns of Varied Antioxidant Enzyme Genes and Nrf2 Signaling Factors.

    Science.gov (United States)

    Wu, Pei; Liu, Yang; Jiang, Wei-Dan; Jiang, Jun; Zhao, Juan; Zhang, Yong-An; Zhou, Xiao-Qiu; Feng, Lin

    2017-01-01

    The liver and intestine are susceptible to the oxidative damage which could result in several diseases. Choline deficiency induced oxidative damage in rat liver cells. Thus, this study aimed to investigate the potential molecular mechanisms responsible for choline deficiency-induced oxidative damage. Juvenile Jian carp were fed diets differing in choline content [165 (deficient group), 310, 607, 896, 1167 and 1820 mg/kg diet] respectively for 65 days. Oxidative damage, antioxidant enzyme activities and related gene expressions in the hepatopancreas and intestine were measured. Choline deficiency decreased choline and phosphatidylcholine contents, and induced oxidative damage in both organs, as evidenced by increased levels of oxidative-stress markers (malondialdehyde, protein carbonyl and 8-hydroxydeoxyguanosine), coupled with decreased activities of antioxidant enzymes [Copper-zinc superoxide dismutase (CuZnSOD), manganese superoxide dismutase (MnSOD), glutathione peroxidase (GPx) and glutathione-S-transferase (GST)]. However, choline deficiency increased glutathione contents in the hepatopancreas and intestine. Furthermore, dietary choline deficiency downregulated mRNA levels of MnSOD, GPx1b, GST-rho, mGST3 and Kelch-like ECH associating protein 1 (Keap1b) in the hepatopancreas, MnSOD, GPx1b, GPx4a, GPx4b, GST-rho, GST-theta, GST-mu, GST-alpha, GST-pi and GST-kappa in the intestine, as well as intestinal Nrf2 protein levels. In contrast, choline deficiency upregulated the mRNA levels of GPx4a, GPx4b, mGST1, mGST2, GST-theta, GST-mu, Keap1a and PKC in the hepatopancreas, mGST3, nuclear factor erythoid 2-related factor 2 (Nrf2) and Keap1a in the intestine, as well as hepatopancreatic Nrf2 protein levels. This study provides new evidence that choline deficiency-induced oxidative damage is associated with changes in the transcription of antioxidant enzyme and Nrf2/Keap1 signaling molecules in the hepatopancreas and intestine. Additionally, this study firstly

  8. Dietary Deficiency of Calcium and/or Iron, an Age-Related Risk Factor for Renal Accumulation of Cadmium in Mice.

    Science.gov (United States)

    Min, Kyong-Son; Sano, Erika; Ueda, Hidenori; Sakazaki, Fumitoshi; Yamada, Keita; Takano, Masaoki; Tanaka, Keiichi

    2015-01-01

    The major route of cadmium (Cd) intake by non-smokers is through food ingestion. Cd is a non-essential metal absorbed through one or more transporters of essential metal ions. Expression of these transporters is affected by nutritional status. To investigate the risk factors for Cd toxicity, the effects of deficiency of essential metals on hepatic and renal accumulation of Cd were studied in mice of different ages. Mice were administered a control diet or one of the essential metal-deficient diets, administered Cd by gavage for 6 weeks, and killed; then, Cd accumulation was evaluated. Iron deficiency (FeDF) or calcium deficiency (CaDF) resulted in remarkable increases in hepatic and renal Cd accumulation compared with control-diet mice and other essential metal-deficient mice. Cd accumulation in hepatic and renal tissue was increased significantly at all ages tested in FeDF and CaDF mice. Renal Cd concentrations were higher in 4-week-old mice than in 8- and 25-week-old mice. Increase in intestinal mRNA expression of calcium transporter (CaT)1, divalent metal ion transporter-1, and metallothionein (MT)1 was also higher in 4-week-old mice than in other mice. Renal accumulation of Cd showed strong correlation with intestinal mRNA expression of CaT1 and MT1. These data suggest that CaDF and FeDF at younger ages can be a risk factor for Cd toxicity.

  9. [Prevalence of vitamin d deficiency and insuffiency and associated factors in pregnant women of northern Spain].

    Science.gov (United States)

    Rodríguez-Dehli, Ana Cristina; Riaño Galán, Isolina; Fernández-Somoano, Ana; Navarrete-Muñoz, Eva María; Espada, Mercedes; Vioque, Jesús; Tardón, Adonina

    2015-04-01

    Introducción y objetivos: El déficit de vitamina D durante el embarazo se ha relacionado con sucesos adversos durante la gestación y con el desarrollo infantil postnatal. En este estudio examinamos los niveles plasmáticos de vitamina D [25(OH)D3] y los factores asociados a su deficiencia e insuficiencia plasmática en embarazadas del norte de España. Material y método: Se han analizado los datos de 453 gestantes participantes en la cohorte INMA-Asturias a las que se determinó la 25(OH)D3 mediante cromatografía líquida de alta resolución. Se ha estimado la ingesta dietética de vitamina D mediante un cuestionario de frecuencia alimentaria validado. Se han estimado las prevalencias de deficiencia [25(OH)D3.

  10. Increased serum and bone matrix levels of transforming growth factor {beta}1 in patients with GH deficiency in response to GH treatment

    DEFF Research Database (Denmark)

    Ueland, Thor; Lekva, Tove; Otterdal, Kari

    2011-01-01

    Patients with adult onset GH deficiency (aoGHD) have secondary osteoporosis, which is reversed by long-term GH substitution. Transforming growth factor β1 (TGFβ1 or TGFB1) is abundant in bone tissue and could mediate some effects of GH/IGFs on bone. We investigated its regulation by GH/IGF1 in vivo...

  11. Application of wide selected-ion monitoring data-independent acquisition to identify tomato fruit proteins regulated by the CUTIN DEFICIENT2 transcription factor

    Science.gov (United States)

    We describe here the use of label-free wide selected-ion monitoring data-independent acquisition (WiSIM-DIA) to identify proteins that are involved in the formation of tomato (Solanum lycopersicum) fruit cuticles and that are regulated by the transcription factor CUTIN DEFICIENT2 (CD2). A spectral l...

  12. Defective jejunal and colonic salt absorption and alteredNa +/H+ exchanger 3 (NHE3) activity in NHE regulatory factor 1 (NHERF1) adaptor protein-deficient mice

    NARCIS (Netherlands)

    N. Broere (Nellie); M. Chen (Min); A. Cinar (Ayhan); A.K. Singh (Arbind); J. Hillesheim (Jutta); B. Riederer (Beat Michel); M. Lunnemann; I. Rottinghaus (Ingrid); A. Krabbenhöft (Anja); R. Engelhardt (Regina); B. Rausch; E.J. Weinman (Edward); M. Donowitz (Mark); A. Hubbard; O. Kocher (Olivier); H.R. de Jonge (Hugo); B.M. Hogema (Boris); U. Seidler (Ursula)

    2009-01-01

    textabstractWe investigated the role of the Na+/H+ exchanger regulatory factor 1 (NHERF1) on intestinal salt and water absorption, brush border membrane (BBM) morphology, and on the NHE3 mRNA expression, protein abundance, and transport activity in the murine intestine. NHERF1-deficient mice display

  13. Skeletal muscle-specific expression of human blood coagulation factor Ⅸ rescues factordeficiency mouse by AAV-mediated gene transfer

    Institute of Scientific and Technical Information of China (English)

    赖立辉; 陈立; 卢大儒; 王琪; 高啸波; 邱信芳; Jerry; L.Hsueh; 薛京伦; 王健民; 周虹

    1999-01-01

    The efficacy of recombinant adeno-associated virus (AAV) vector to deliver and express human blood clotting factor DC (hFIX) gene in skeletal muscle of coagulation factor IX deficiency mouse strain (FactorIX-knockout) is e-valuated. The muscle creatine kinase enhancer (MCK) and βactin promoter ((3A) were used to drive the hFIX minigene (hFIXml), which was flanked by AAV inverted terminal repeats (ITRs). Following intramuscular injection of high liter (2.5 x 1011 vector genomes/mL) of AAV, increased hFIX expression (256 ng/mL of plasma) was achieved. The time course of hFIX expression demonstrated that the expression level gradually increased over a period of two weeks before anti-hFIX antibodies developed in mouse circulating plasma. Those results provided a promising evidence that rAAV-me-diated gene transfer and skeletal muscle-specific expression of hFIX is a feasible strategy for treating patients for hemophilia B.

  14. Micronutrient deficiencies as predisposing factors for hypertension in lacto-vegetarian Indian adults.

    Science.gov (United States)

    Chiplonkar, Shashi A; Agte, Vaishali V; Tarwadi, Kirtan V; Paknikar, Kishor M; Diwate, Uma P

    2004-06-01

    With the increasing knowledge about the antioxidant potential of many micronutrients such as zinc and vitamin C, their roles in oxidative stress related health disorders have been postulated. This study therefore investigated low micronutrient status as a predisposing factor for hypertension in a traditionally lacto-vegetarian population like Indians. Micronutrient profile was assessed in 109 hypertensives with age-gender-socio-economic status matched 115 healthy normotensives (30-58 years of age). Food intakes were estimated through a semi-quantitative food frequency questionnaire. Nutrient intakes were then evaluated by previous estimates of cooked foods from our laboratory. Systolic and diastolic blood pressure (SBP, DBP), age, weight, height, waist and hip circumference, occupation, physical activity, smoking habits were recorded. Fasting blood samples were analyzed for hemoglobin, serum level of glucose, triglycerides, total cholesterol, HDL, ceruloplasmin, plasma level of ascorbic acid, folic acid, retinol, erythrocyte glutathione reductase activity coefficient (EGRAC) and erythrocyte membrane zinc. There were no significant differences between protein, fat intakes of normal and hypertensive individuals, though intakes of men were higher than those of women (p 0.2) in these subjects. Conditional logistic regression analysis indicated that intakes of vitamin C, folic acid and zinc were associated with 18% (OR = 1.18, 95% CI:1.08, 1.26), 51% (OR = 1.51, 95% CI 0.94, 2.1) higher odds for hypertension, and 3% lower odds for hypertension (OR = 0.97, 95% CI 0.92, 1.01), respectively. Mean plasma vitamin C and folic acid were significantly higher (p < 0.01), and serum ceruloplasmin and erythrocyte membrane zinc were marginally higher (p = 0.07) in normal than hypertensive subjects. In multivariate linear regression analyses, plasma vitamin C, serum ceruloplasmin and erythrocyte membrane zinc were negatively associated with SBP (p = 0.00001) and plasma vitamin C was

  15. Anemia and iron deficiency among school adolescents: burden, severity, and determinant factors in southwest Ethiopia

    Science.gov (United States)

    Tesfaye, Melkam; Yemane, Tilahun; Adisu, Wondimagegn; Asres, Yaregal; Gedefaw, Lealem

    2015-01-01

    Background Adolescence is the period of most rapid growth second to childhood. The physical and physiological changes that occur in adolescents place a great demand on their nutritional requirements and make them more vulnerable to anemia. Anemia in the adolescence causes reduced physical and mental capacity and diminished concentration in work and educational performance, and also poses a major threat to future safe motherhood in girls. The purpose of this study was to determine the prevalence of anemia and its associated factors among school adolescents in Bonga Town, southwest Ethiopia. Methods A cross-sectional study was conducted among 408 school adolescents in Bonga Town, southwest Ethiopia, from March 15, 2014 to May 25, 2014. An interviewer-administered questionnaire was used to collect sociodemographic and other data. A total of 7 mL of venous blood and 4 g of stool samples were collected from each study participant. Blood and stool samples were analyzed for hematological and parasitological analyses, respectively. Data were analyzed using SPSS Version 20 software for Windows. Results The overall prevalence of anemia was 15.2% (62/408), of which 83.9% comprised mild anemia. The proportion of microcytic, hypochromic anemia was 53% (33/62). Being female (adjusted odds ratio [AOR] =3.04, 95% confidence interval (CI) =1.41–6.57), household size ≥5 (AOR =2.58, 95% CI =1.11–5.96), father’s illiteracy (AOR =9.03, 95% CI =4.29–18.87), intestinal parasitic infection (AOR =5.37, 95% CI =2.65–10.87), and low body mass index (AOR =2.54, 95% CI =1.17–5.51) were identified as determinants of anemia among school adolescents. Conclusion This study showed that anemia was a mild public health problem in this population. School-based interventions on identified associated factors are important to reduce the burden of anemia among school adolescents. PMID:26719736

  16. Brain-derived neurotrophic factor-deficient mice exhibit a hippocampal hyperserotonergic phenotype.

    Science.gov (United States)

    Guiard, Bruno P; David, Denis J P; Deltheil, Thierry; Chenu, Franck; Le Maître, Erwan; Renoir, Thibault; Leroux-Nicollet, Isabelle; Sokoloff, Pierre; Lanfumey, Laurence; Hamon, Michel; Andrews, Anne M; Hen, René; Gardier, Alain M

    2008-02-01

    Growing evidence supports the involvement of brain-derived neurotrophic factor (BDNF) in mood disorders and the mechanism of action of antidepressant drugs. However, the relationship between BDNF and serotonergic signalling is poorly understood. Heterozygous mutants BDNF +/- mice were utilized to investigate the influence of BDNF on the serotonin (5-HT) system and the activity of the serotonin transporter (SERT) in the hippocampus. The zero net flux method of quantitative microdialysis revealed that BDNF +/- heterozygous mice have increased basal extracellular 5-HT levels in the hippocampus and decreased 5-HT reuptake capacity. In keeping with these results, the selective serotonin reuptake inhibitor paroxetine failed to increase hippocampal extracellular 5-HT levels in BDNF +/- mice while it produced robust effects in wild-type littermates. Using in-vitro autoradiography and synaptosome techniques, we investigated the causes of attenuated 5-HT reuptake in BDNF +/- mice. A significant decrease in [3H]citalopram-binding-site density in the CA3 subregion of the ventral hippocampus and a significant reduction in [3H]5-HT uptake in hippocampal synaptosomes, revealed mainly a decrease in SERT function. However, 5-HT1A autoreceptors were not desensitized in BDNF +/- mice. These results provide evidence that constitutive reductions in BDNF modulate SERT function reuptake in the hippocampus.

  17. Effect of iron deficiency on the expression of insulin-like growth factor-II and its receptor in neuronal and glial cells.

    Science.gov (United States)

    Morales González, E; Contreras, I; Estrada, J A

    2014-09-01

    Many studies have demonstrated that iron deficiency modifies the normal function of the central nervous system and alters cognitive abilities. When cellular damage occurs in the central nervous system, neuroprotective mechanisms, such as the production of neurotrophic factors, are essential in order for nervous tissue to function correctly. Insulin-like growth factor II (IGF- II) is a neurotrophic factor that was recently shown to be involved in the normal functioning of cognitive processes in animal models. However, the impact of iron deficiency on the expression and function of this molecule has not yet been clarified. Mixed primary cell cultures from the central nervous system were collected to simulate iron deficiency using deferoxamine. The expression of IGF-I, IGF-II, IGF-IR, and IGF-IIR was determined with the western blot test. We observed increased expression of IGF-II, along with a corresponding decrease in the expression of IGF-IIR, in iron-deficient mixed primary cell cultures. We did not observe alterations in the expression of these proteins in isolated microglia or neuronal cultures under the same conditions. We did not detect differences in the expression of IGF-I and IGF-IR in iron-deficient cultures. In vitro iron deficiency increases the expression of IGF-II in mixed glial cell cultures, which may have a beneficial effect on brain tissue homeostasis in a situation in which iron availability is decreased. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  18. Altered Responses to Cold Environment in Urocortin 1 and Corticotropin-Releasing Factor Deficient Mice

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    Bayan Chaker

    2013-01-01

    Full Text Available We examined core body temperature (CBT of urocortin 1 (UCN1 and corticotropin releasing factor (CRF knockout (KO mice exposed to 4°C for 2 h. UCN1KO mice showed higher average CBT during cold exposure as compared to WT. The CBT of male and female WT mice dropped significantly to 34.1 ± 2.4 and 34.9 ± 3.1 C at 4°C, respectively. In contrast, the CBT of male and female UCN1KO mice dropped only slightly after 2 h at 4°C to 36.8 ± 0.7 and 38.1 ± 0.5 C, respectively. WT female and male UCN1KO mice showed significant acclimatization to cold; however, female UCN1KO mice did not show such a significant acclimatization. CRFKO mice showed a dramatic decline in CBT from 38.2 ±  0.4 at 22°C to 26.1 ± 9.8 at 4°C for 2 h. The CRF/UCN1 double KO (dKO mice dropped their CBT to 32.5 ± 4.0 after 2 h exposure to 4°C. Dexamethasone treatment prevented the decline in CBT of the CRFKO and the dKO mice. Taken together, the data suggest a novel role for UCN1 in thermoregulation. The role of CRF is likely secondary to adrenal glucocorticoids, which have an important regulatory role on carbohydrate, fat, and protein metabolism.

  19. Prevalence and risk factors for vitamin C deficiency in north and south India: a two centre population based study in people aged 60 years and over.

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    Ravilla D Ravindran

    Full Text Available BACKGROUND: Studies from the UK and North America have reported vitamin C deficiency in around 1 in 5 men and 1 in 9 women in low income groups. There are few data on vitamin C deficiency in resource poor countries. OBJECTIVES: To investigate the prevalence of vitamin C deficiency in India. DESIGN: We carried out a population-based cross-sectional survey in two areas of north and south India. Randomly sampled clusters were enumerated to identify people aged 60 and over. Participants (75% response rate were interviewed for tobacco, alcohol, cooking fuel use, 24 hour diet recall and underwent anthropometry and blood collection. Vitamin C was measured using an enzyme-based assay in plasma stabilized with metaphosphoric acid. We categorised vitamin C status as deficient (28 µmol/L. We investigated factors associated with vitamin C deficiency using multivariable Poisson regression. RESULTS: The age, sex and season standardized prevalence of vitamin C deficiency was 73.9% (95% confidence Interval, CI 70.4,77.5 in 2668 people in north India and 45.7% (95% CI 42.5,48.9 in 2970 from south India. Only 10.8% in the north and 25.9% in the south met the criteria for adequate levels. Vitamin C deficiency varied by season, and was more prevalent in men, with increasing age, users of tobacco and biomass fuels, in those with anthropometric indicators of poor nutrition and with lower intakes of dietary vitamin C. CONCLUSIONS: In poor communities, such as in our study, consideration needs to be given to measures to improve the consumption of vitamin C rich foods and to discourage the use of tobacco.

  20. Iron deficiency, an independent risk factor for isolated hypothyroxinemia in pregnant and nonpregnant women of childbearing age in China.

    Science.gov (United States)

    Yu, Xiaohui; Shan, Zhongyan; Li, Chenyan; Mao, Jinyuan; Wang, Weiwei; Xie, Xiaochen; Liu, Aihua; Teng, Xiaochun; Zhou, Weiwei; Li, Chenyang; Xu, Bin; Bi, Lihua; Meng, Tao; Du, Jianling; Zhang, Shaowei; Gao, Zhengnan; Zhang, Xiaomei; Yang, Liu; Fan, Chenling; Teng, Weiping

    2015-04-01

    Isolated hypothyroxinemia during early pregnancy may irreversibly damage the neurodevelopment of offspring. However, the causes are not well clarified. To explore the association of iron deficiency (ID) with hypothyroid function of women in early pregnancy and nonpregnant women. A total of 7953 pregnant women of ≤ 12 weeks gestation and 2000 childbearing-aged women were recruited. A subpopulation including 3340 pregnant women and 1052 nonpregnant women with sufficient iodine intake and negative thyroid peroxidase antibody were studied. Mild and severe cases of hypothyroxinemia were defined as free T4 levels below the 10th percentile and the 5th percentile, respectively, with normal TSH. Total body iron, serum ferritin, and serum transferrin receptor were used as indicators for iron nutrition. Serum free T4 levels were significantly lower in both pregnant and nonpregnant women with ID compared with the corresponding groups without ID (both P women with ID than those without, in both pregnant and nonpregnant women (all P pregnant and nonpregnant childbearing-aged women, independent of the effects of iodine and thyroid autoimmunity. We speculate that ID may be a pathogenic factor for hypothyroxinemia, even in pregnant women during the first trimester.

  1. A synthetic interaction screen identifies factors selectively required for proliferation and TERT transcription in p53-deficient human cancer cells.

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    Li Xie

    Full Text Available Numerous genetic and epigenetic alterations render cancer cells selectively dependent on specific genes and regulatory pathways, and represent potential vulnerabilities that can be therapeutically exploited. Here we describe an RNA interference (RNAi-based synthetic interaction screen to identify genes preferentially required for proliferation of p53-deficient (p53- human cancer cells. We find that compared to p53-competent (p53+ human cancer cell lines, diverse p53- human cancer cell lines are preferentially sensitive to loss of the transcription factor ETV1 and the DNA damage kinase ATR. In p53- cells, RNAi-mediated knockdown of ETV1 or ATR results in decreased expression of the telomerase catalytic subunit TERT leading to growth arrest, which can be reversed by ectopic TERT expression. Chromatin immunoprecipitation analysis reveals that ETV1 binds to a region downstream of the TERT transcriptional start-site in p53- but not p53+ cells. We find that the role of ATR is to phosphorylate and thereby stabilize ETV1. Our collective results identify a regulatory pathway involving ETV1, ATR, and TERT that is preferentially important for proliferation of diverse p53- cancer cells.

  2. DIAGNOSTIC VALUE OF SERUM INSULIN-LIKE GROWTH FACTOR BINDING PROTEIN-3 IN CHILDREN WITH OR WITHOUT GROWTH HORMONE DEFICIENCY

    Institute of Scientific and Technical Information of China (English)

    覃舒文; 史轶蘩; 邓洁英

    2002-01-01

    Objective. To study the value of serum insulin-like growth factor binding protein-3 (IGFBP-3) levels in differential diagnosis of growth hormone deficiency (GHD). Methods. To measure serum IGFBP-3 levels by RIA in normal children and adolescents, GHD children and short-stature children without GHD. Results. Serum level of IGFBP-3 in 129 children with untreated GHD and with no pubertal development was 1.6± 0.9 mg/L, which was less than that in normal group of the same age, but overlapped with the normal children in Tanner stage I. After six -month treatment with recombinant human growth hormone (rhGH), serum level of IGFBP-3 in 59 GHD significantly increased from 1.3± 0.7 mg/L to 2.7± 0.9 mg/L, accompanied by an increase of body heights, growth velocities and serum level of IGF-1. Serum level of IGFBP-3 in 55 short-stature children without GHD was 3.3± 2.2 mg/L, which was not significantly different from that in normal group. Conclusion. Serum IGFBP-3 level can reflect the status of GH secretion in children with GHD and is a useful marker for differential diagnosis of GHD.

  3. DIAGNOSTIC VALUE OF SERUM INSULIN—LIKE GROWTH FACTOR BINDING PROTEIN—3 IN CHILDREN WITH OR WITHOUT GROWTH HORMONE DEFICIENCY

    Institute of Scientific and Technical Information of China (English)

    覃舒文; 史轶蘩; 等

    2002-01-01

    Objective:To study the value of serum insulin-like growth factor binding protein-3 (IGFBP-3) levels in differential diagnosis of growth hormone deficiency(GHD).Methods:To measure serum IGFBP-3 levels by RIA in normal children and adolescents,GHD children and short-stature children without (GHD).Methods:To measure serum IGFBP-3 levels by RIA in normal children and adolescents,GHD children and short-stature children without GHD.Results.Serum level of IGFBP-3 in 129 children with untreated GHD and with no pubertal development was 1.6±0.9mg/L,which was less than that in normal group of the same age,but overlapped with the normal children in Tanner stage I.After six-month treatment with recombinant human growth hormone(rhGH),serum level of IGFBP-3 in 59 GHD significantly increased from 1.3±0.7mg/L to 2.7±0.9mg/L,accompanied by an increase of body heights,growth velocities and serum level of IGF-1.Serum level of IGFBP-3 in 55 shortstature children without GHD was 3.3±2.2mg/L,which was not significantly different from that in normal group.Conclusion:Serum IGFBP-3 level can reflect the status of GH secretion in children with GHD and is a useful marker for differential diagnosis of GHD.

  4. Apoptosis-inducing factor deficiency decreases the proliferation rate and protects the subventricular zone against ionizing radiation.

    Science.gov (United States)

    Osato, K; Sato, Y; Ochiishi, T; Osato, A; Zhu, C; Sato, M; Swanpalmer, J; Modjtahedi, N; Kroemer, G; Kuhn, H G; Blomgren, K

    2010-10-21

    Cranial radiotherapy in children often leads to progressive cognitive decline. We have established a rodent model of irradiation-induced injury to the young brain. A single dose of 8 Gy was administered to the left hemisphere of postnatal day 10 (P10) mice. Harlequin (Hq) mice, carrying the hypomorphic apoptosis-inducing factor AIF(Hq) mutation, express 60% less AIF at P10 and displayed significantly fewer dying cells in the subventricular zone (SVZ) 6 h after IR, compared with wild type (Wt) littermates. Irradiated cyclophilin A-deficient (CypA(-/-)) mice confirmed that CypA has an essential role in AIF-induced apoptosis after IR. Hq mice displayed no reduction in SVZ size 7 days after IR, whereas 48% of the SVZ was lost in Wt mice. The proliferation rate was lower in the SVZ of Hq mice. Cultured neural precursor cells from the SVZ of Hq mice displayed a slower proliferation rate and were more resistant to IR. IR preferentially kills proliferating cells, and the slower proliferation rate in the SVZ of Hq mice may, at least partly, explain the protective effect of the Hq mutation. Together, these results indicate that targeting AIF may provide a fruitful strategy for protection of normal brain tissue against the detrimental side effects of IR.

  5. Clinical decision-making for vitamin K-1 and K-2 deficiency and coronary artery calcification with warfarin therapy: are diet, factor Xa inhibitors or both the answer?

    Science.gov (United States)

    Wahlqvist, Mark L; Tanaka, Kiyoshi; Tzeng, Bing-Hsiean

    2013-01-01

    Coronary artery calcification is a recognised risk factor for ischaemic heart disease and mortality. Evidence is now strong that Mönckeberg's arteriosclerosis, a form of vascular calcification, can be attributable to vitamin K deficiency, but that vitamin K-2, especially the MK-4 form from foods like cheese can be protective. Warfarin blocks the recycling of hepatic and peripheral vitamin K leading to secondary vitamin K deficiency with adverse effects on vasculature, bone, kidneys, brain and other tissues and systems (inflammatory, immune function and neoplasia at least). There is individual susceptibility to vitamin K deficiency and warfarin sensitivity, partly explicable in terms of genetic polymorphisms, epigenetics, diet and pharmacotherapy. The emergence of extensive coronary calcification in a man with atrial fibrillation treated for a decade with warfarin is described by way of illustration and to raise the present clinical management conundrums. Finally, a putative set of recommendations is provided.

  6. Granulocyte Colony Stimulating Factor and MT1-MMP Involved in Development of Atherosclerosis in Apolipoprotein E-Deficient Mice

    Institute of Scientific and Technical Information of China (English)

    Su-zhen GUO; Andres J. Espinoza; Christian A. Espinoza; Terence M. Doherty; Xiao WANG

    2009-01-01

    Objectives Genetic deficiency of macrophage colony stimulating factor (M-CSF) in atherosclerosis-prone (apoE-/-) mice markedly reduces formation of atheroma. But Little is known about the potential effects of other colony stimulating factors(CSF), such as granulocyte CSF(G-CSF), on atherosclerosis. This study tested the hypothesis that G-CSF would be involved in development of atherosclerotic plaque. Methods apoE-/- mice fed with a Western-style diet (0.15% cholesterol) were injected subcutaneously with recombinant human G-CSF(10 mg/day) daily for 9 weeks then sacrificed. The matrix metalloproteinase(MMP)2 and MMP9 in serum of mice were measured by Gelatin Zymography analysis and c-kit and membrane type1-MMP(MT1-MMP) antigens were detected using fluorescence activated cell sorting (FACS). Meanwhile, complete blood counts (CBC) and serum cholesterol, relative fractions of VLDL,LDL, and HDL were evaluated by spectrophotometric techniques and high performance liquid chromatography (HPLC)respectively. Atherosclerotic Lesions of the aorta were also analyzed by histological methods. Results G-CSF treatment resulted in increased proportions of circulating monocytes (6.9±2.2% vs.3.8±0.3%;P<0.05), and decreased serum levels of total cholesterol (1225±594 vs.1991±1009;P<0.005) compared to control mice. A greater proportion of bone marrow cells from G-CSF treated mice expressed MT1-MMP (14.5±5.5% vs.6.2±5.0%, P<0.05) compared to bone marrow cells from vehicle treated mice. G-CSF treatment was also associated with smaller atheromatous plaque, and decreased oil red O staining. Conclusions G-CSF lowers serum cholesterol, increases circulating monocytes, increases bone marrow cell expression of MT1-MMP, inhibits plaque development, and decreases lipid and macrophage infiltration into developing plaque.

  7. Interferon regulatory factor 8-deficiency determines massive neutrophil recruitment but T cell defect in fast growing granulomas during tuberculosis.

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    Stefano Rocca

    Full Text Available Following Mycobacterium tuberculosis (Mtb infection, immune cell recruitment in lungs is pivotal in establishing protective immunity through granuloma formation and neogenesis of lymphoid structures (LS. Interferon regulatory factor-8 (IRF-8 plays an important role in host defense against Mtb, although the mechanisms driving anti-mycobacterial immunity remain unclear. In this study, IRF-8 deficient mice (IRF-8⁻/⁻ were aerogenously infected with a low-dose Mtb Erdman virulent strain and the course of infection was compared with that induced in wild-type (WT-B6 counterparts. Tuberculosis (TB progression was examined in both groups using pathological, microbiological and immunological parameters. Following Mtb exposure, the bacterial load in lungs and spleens progressed comparably in the two groups for two weeks, after which IRF-8⁻/⁻ mice developed a fatal acute TB whereas in WT-B6 the disease reached a chronic stage. In lungs of IRF-8⁻/⁻, uncontrolled growth of pulmonary granulomas and impaired development of LS were observed, associated with unbalanced homeostatic chemokines, progressive loss of infiltrating T lymphocytes and massive prevalence of neutrophils at late infection stages. Our data define IRF-8 as an essential factor for the maintenance of proper immune cell recruitment in granulomas and LS required to restrain Mtb infection. Moreover, IRF-8⁻/⁻ mice, relying on a common human and mouse genetic mutation linked to susceptibility/severity of mycobacterial diseases, represent a valuable model of acute TB for comparative studies with chronically-infected congenic WT-B6 for dissecting protective and pathological immune reactions.

  8. Interferon regulatory factor 8-deficiency determines massive neutrophil recruitment but T cell defect in fast growing granulomas during tuberculosis.

    Science.gov (United States)

    Rocca, Stefano; Schiavoni, Giovanna; Sali, Michela; Anfossi, Antonio Giovanni; Abalsamo, Laura; Palucci, Ivana; Mattei, Fabrizio; Sanchez, Massimo; Giagu, Anna; Antuofermo, Elisabetta; Fadda, Giovanni; Belardelli, Filippo; Delogu, Giovanni; Gabriele, Lucia

    2013-01-01

    Following Mycobacterium tuberculosis (Mtb) infection, immune cell recruitment in lungs is pivotal in establishing protective immunity through granuloma formation and neogenesis of lymphoid structures (LS). Interferon regulatory factor-8 (IRF-8) plays an important role in host defense against Mtb, although the mechanisms driving anti-mycobacterial immunity remain unclear. In this study, IRF-8 deficient mice (IRF-8⁻/⁻) were aerogenously infected with a low-dose Mtb Erdman virulent strain and the course of infection was compared with that induced in wild-type (WT-B6) counterparts. Tuberculosis (TB) progression was examined in both groups using pathological, microbiological and immunological parameters. Following Mtb exposure, the bacterial load in lungs and spleens progressed comparably in the two groups for two weeks, after which IRF-8⁻/⁻ mice developed a fatal acute TB whereas in WT-B6 the disease reached a chronic stage. In lungs of IRF-8⁻/⁻, uncontrolled growth of pulmonary granulomas and impaired development of LS were observed, associated with unbalanced homeostatic chemokines, progressive loss of infiltrating T lymphocytes and massive prevalence of neutrophils at late infection stages. Our data define IRF-8 as an essential factor for the maintenance of proper immune cell recruitment in granulomas and LS required to restrain Mtb infection. Moreover, IRF-8⁻/⁻ mice, relying on a common human and mouse genetic mutation linked to susceptibility/severity of mycobacterial diseases, represent a valuable model of acute TB for comparative studies with chronically-infected congenic WT-B6 for dissecting protective and pathological immune reactions.

  9. Nutritional iron deficiency

    NARCIS (Netherlands)

    Zimmermann, M.B.; Hurrell, R.F.

    2007-01-01

    Iron deficiency is one of the leading risk factors for disability and death worldwide, affecting an estimated 2 billion people. Nutritional iron deficiency arises when physiological requirements cannot be met by iron absorption from diet. Dietary iron bioavailability is low in populations consuming

  10. Nutritional iron deficiency

    NARCIS (Netherlands)

    Zimmermann, M.B.; Hurrell, R.F.

    2007-01-01

    Iron deficiency is one of the leading risk factors for disability and death worldwide, affecting an estimated 2 billion people. Nutritional iron deficiency arises when physiological requirements cannot be met by iron absorption from diet. Dietary iron bioavailability is low in populations consuming

  11. Pathogenesis of thyroid-associated ophthalmopathy: does autoimmunity against calsequestrin and collagen XIII play a role?

    Directory of Open Access Journals (Sweden)

    Hooshang Lahooti

    2010-04-01

    Full Text Available Hooshang Lahooti, Kishan R Parmar, Jack R WallThe Department of Medicine, University of Sydney, Nepean Clinical School, Penrith, NSW, AustraliaAbstract: Thyroid-associated ophthalmopathy (TAO, or thyroid eye disease, is a complex inflammatory disorder of the eye that, as its name implies, is associated with thyroid disease. TAO can be divided into three subtypes: ocular myopathy, congestive myopathy and mixed congestive and myopathic ophthalmopathy. Although the precise pathophysiology of TAO remains unclear it is likely to reflect an autoimmune reaction involving sensitized T-cells and autoantibodies directed against a thyroid and orbital tissue shared antigen. One well studied candidate in this immune reaction is the thyroid-stimulating hormone receptor (TSH-r, expressed in the orbital fibroblast and pre adipocyte. In our studies of TAO, we have investigated the nature and significance of antibodies targeting other eye muscle and orbital connective tissue (OCT antigens. Our findings suggest that autoimmunity against the eye muscle antigen calsequestrin and the OCT antigen collagen XIII plays a role in the pathogenesis of TAO. We propose that ocular myopathy and chronic eyelid retraction are due to autoimmunity against skeletal muscle calsequestrin in the extraocular and eyelid muscles, respectively. This may be initiated in the thyroid where calsequestrin expression is upregulated, possibly due to a stimulatory effect of TSH-r antibodies. We also propose that congestive ophthalmopathy results from a reaction against the TSH-r or collagen XIII in orbital fibroblast cell membranes. Further insight into the role of eye muscle and OCT antigens in the pathogenesis of TAO may allow for the development of new therapies to treat the eye disorder and reduce patient morbidity.Keywords: thyroid-associated ophthalmopathy, autoimmunity, t calsequestrin, collagen XIII

  12. Micronutrient Deficiencies and Related Factors in School-Aged Children in Ethiopia: A Cross-Sectional Study in Libo Kemkem and Fogera Districts, Amhara Regional State

    Science.gov (United States)

    Herrador, Zaida; Sordo, Luis; Gadisa, Endalamaw; Buño, Antonio; Gómez-Rioja, Rubén; Iturzaeta, Jose Manuel; de Armas, Lisset Fernandez; Benito, Agustín; Aseffa, Abraham; Moreno, Javier; Cañavate, Carmen; Custodio, Estefania

    2014-01-01

    Introduction The present study describes the distribution of selected micronutrients and anaemia among school-aged children living in Libo Kemkem and Fogera (Amhara State, Ethiopia), assessing differences by socio-demographic characteristics, health status and dietary habits. Methods A cross-sectional survey was carried out during May–December 2009. Socio-demographic characteristics, health status and dietary habits were collected. Biomarkers were determined for 764 children. Bivariate and multivariable statistical methods were employed to assess micronutrient deficiencies (MD), anaemia, and their association with different factors. Results More than two thirds of the school-aged children (79.5%) had at least one MD and 40.5% had two or more coexisting micronutrient deficiencies. The most prevalent deficiencies were of zinc (12.5%), folate (13.9%), vit A (29.3%) and vit D (49%). Anaemia occurred in 30.9% of the children. Children living in rural areas were more likely to have vit D insufficiency [OR: 5.9 (3.7–9.5)] but less likely to have folate deficiency [OR: 0.2 (0.1–0.4)] and anaemia [OR: 0.58 (0.35–0.97)]. Splenomegaly was positively associated with folate deficiency and anaemia [OR: 2.77 (1.19–6.48) and 4.91 (2.47–9.75)]. Meat and fish consumption were inversely correlated with zinc and ferritin deficiencies [OR: 0.2 (0.1–0.8) and 0.2 (0.1–0.9)], while oil consumption showed a negative association with anaemia and deficiencies of folate and vitamin A [0.58 (0.3–0.9), OR: 0.5 (0.3–0.9) and 0.6 (0.4–0.9)]. Serum ferritin levels were inversely correlated to the presence of anaemia (p<0.005). Conclusion There is a high prevalence of vitamin A deficiency and vitamin D insufficiency and a moderate prevalence of zinc and folate deficiencies in school-aged children in this area. The inverse association of anaemia and serum ferritin levels may be due to the presence of infectious diseases in the area. To effectively tackle malnutrition

  13. Lethal factor VII deficiency due to novel mutations in the F7 promoter: functional analysis reveals disruption of HNF4 binding site.

    Science.gov (United States)

    Giansily-Blaizot, Muriel; Lopez, Estelle; Viart, Victoria; Chafa, Ouerdia; Tapon-Bretaudière, Jacqueline; Claustres, Mireille; Taulan, Magali

    2012-08-01

    Hereditary factor VII (FVII) deficiency is a rare autosomal recessive disorder. Deleterious mutations that prevent the synthesis of any amount of functional FVII have been associated with life-threatening haemorrhage in neonates. Here we report two infants, of Maghrebian origin, who suffered a fatal spontaneous cerebral haemorrhage. Investigation of the molecular basis for their severe FVII deficiency revealed novel mutations in a homozygous state within the F7 gene promoter: a single nucleotide substitution (c.-65G>C) and a 2bp deletion (c.-60_-59delTT). To determine whether these promoter variants were responsible for the FVII deficiency, computer-assisted sequence analyses were performed. The data predicted a disrupted binding of both HNF4 and COUP-TF transcription factors with each variant. Concordantly, experimental results revealed an altered HNF4-induced transactivation in the promoter mutated variants. The execution of functional tests is critical to ensuring a complete understanding of the effect of any promoter mutant on FVII deficiency. Only then can an accurate molecular diagnosis be made and further genetic counselling and prenatal diagnosis be offered.

  14. Glucose-6-phosphate-dehydrogenase deficiency and its correlation with other risk factors in jaundiced newborns in Southern Brazil

    Institute of Scientific and Technical Information of China (English)

    Clarissa Gutirrez Carvalho; Simone Martins Castro; Ana Paula Santin; Carina Zaleski; Felipe Gutirrez Carvalho; Roberto Giugliani

    2011-01-01

    Objective:To evaluate the correlation between glucose-6-phosphate-dehydrogenase (G6PD) deficiency and neonatal jaundice.Methods: Prospective, observational case-control study was conducted on490 newborns admitted to Hospital de Clínicas de Porto Alegre for phototherapy, who all experienced35 or more weeks of gestation, from March to December2007. Enzymatic screening ofG6PD activity was performed, followed byPCR.Results:There was prevalence of4.6% and a boy-girl ratio of3:1 in jaundiced newborns. No jaundiced neonate withABO incompatibility presented G6PD deficiency, and no Mediterranean mutation was found. A higher proportion of deficiency was observed in Afro-descendants. There was no association withUGT1A1 variants. Conclusions:G6PD deficiency is not related to severe hyperbilirubinemia and considering the high miscegenation in this area of Brazil, other gene interactions should be investigated.

  15. Los milagros en la religiosidad hispánica (siglos XIII al XVI

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    Gerardo Rodríguez

    2009-01-01

    Full Text Available Entre los siglos XIII a XVI, la Península Ibérica manifestó una viva presencia del hecho milagroso en la realidad cotidiana ; esta presencia se expresó en la religiosidad popular y quedó registrada en las diferentes plasmaciones escritas de lo milagroso, desde las obras de Gonzalo de Berceo, Alfonso X y Pero Marín al corpus documental conservado en el Archivo del Real Monasterio de Guadalupe .Estos textos permiten tanto percibir las creencias y prácticas religiosas de dichos siglos como estab...

  16. XIII Edición del Programa Redes: Estudio y valoración

    OpenAIRE

    Tortosa Ybáñez, María Teresa; Álvarez Teruel, José Daniel; Pellín Buades, Neus

    2015-01-01

    El Programa Redes de Investigación en Docencia Universitaria que gestiona y coordina el Vicerrectorado de Estudios, Formación y Calidad y el Instituto de Ciencias de la Educación (ICE) de la Universidad de Alicante en su XIII Convocatoria, ha tenido como objeto el contribuir al impulso y consolidación de equipos ya comprometidos en iniciativas de innovación e investigación en la enseñanza universitaria y promover su experiencia para generar, ampliar, difundir e intercambiar el conocimiento en...

  17. A novel NAC transcription factor, IDEF2, that recognizes the iron deficiency-responsive element 2 regulates the genes involved in iron homeostasis in plants.

    Science.gov (United States)

    Ogo, Yuko; Kobayashi, Takanori; Nakanishi Itai, Reiko; Nakanishi, Hiromi; Kakei, Yusuke; Takahashi, Michiko; Toki, Seiichi; Mori, Satoshi; Nishizawa, Naoko K

    2008-05-09

    Iron is essential for most living organisms, and thus iron deficiency poses a major abiotic stress in crop production. Plants induce iron utilization systems under conditions of low iron availability, but the molecular mechanisms of gene regulation under iron deficiency remain largely unknown. We identified a novel transcription factor of rice and barley, IDEF2, which specifically binds to the iron deficiency-responsive cis-acting element 2 (IDE2) by yeast one-hybrid screening. IDEF2 belongs to an uncharacterized branch of the NAC transcription factor family and exhibits novel properties of sequence recognition. An electrophoretic mobility shift assay and cyclic amplification and selection of targets experiment revealed that IDEF2 predominantly recognized CA(A/C)G(T/C)(T/C/A)(T/C/A) within IDE2 as the core-binding site. IDEF2 transcripts are constitutively present in rice roots and leaves. Repression of the function of IDEF2 by the RNA interference (RNAi) technique and chimeric repressor gene-silencing technology (CRES-T) caused aberrant iron homeostasis in rice. Several genes up-regulated by iron deficiency, including the Fe(II)-nicotianamine transporter gene OsYSL2, were less induced by iron deficiency in the RNAi rice of IDEF2, suggesting that IDEF2 is involved in the regulation of these genes. Many genes with repressed expression in IDEF2 RNAi rice possessed the IDEF2-binding core sites in their promoters, and the flanking sequences were also highly homologous to IDE2. IDEF2 bound to OsYSL2 promoter region containing the binding core site, suggesting direct regulation of OsYSL2 expression. These results reveal novel cis-element/trans-factor interactions functionally associated with iron homeostasis.

  18. A deficiency of apoptosis inducing factor (AIF in Harlequin mouse heart mitochondria paradoxically reduces ROS generation during ischemia-reperfusion

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    Qun eChen

    2014-07-01

    Full Text Available Background and Aims: AIF (apoptosis inducing factor is a flavin and NADH containing protein located within mitochondria required for optimal function of the respiratory chain. AIF may function as an antioxidant within mitochondria, yet when released from mitochondria it activates caspase-independent cell death. The Harlequin (Hq mouse has a markedly reduced content of AIF, providing an experimental model to query if the main role of AIF in the exacerbation of cell death is enhanced mitochondrial generation of reactive oxygen species (ROS or the activation of cell death programs. We asked if the ROS generation is altered in Hq heart mitochondria at baseline or following ischemia-reperfusion (IR.Methods: Buffer perfused mouse hearts underwent 30 min ischemia and 30 min reperfusion. Mitochondrial function including oxidative phosphorylation and H2O2 generation was measured. Immunoblotting was used to determine the contents of AIF and PAR [poly(ADP-ribose] in cell fractions.Results: There were no differences in the release of H2O2 between wild type (WT and Hq heart mitochondria at baseline. IR increased H2O2 generation from WT but not from Hq mitochondria compared to corresponding time controls. The complex I activity was decreased in WT but not in Hq mice following IR. The relocation of AIF from mitochondria to nucleus was increased in WT but not in Hq mice. IR activated PARP-1 only in WT mice. Cell injury was decreased in Hq mouse heart following in vitro IR.Conclusion: A deficiency of AIF within mitochondria does not increase ROS production during IR, indicating that AIF functions less as an antioxidant within mitochondria. The decreased cardiac injury in Hq mouse heart accompanied by less AIF translocation to the nucleus suggests that AIF relocation, rather than the AIF content within mitochondria, contributes to cardiac injury during IR.

  19. Is hyperuricemia a risk factor for arteriosclerosis? Uric acid and arteriosclerosis in apolipoprotein e-deficient mice.

    Science.gov (United States)

    Wakuda, Hirokazu; Uchida, Shinya; Ikeda, Masahiko; Tabuchi, Masaki; Akahoshi, Yasumitsu; Shinozuka, Kazumasa; Yamada, Shizuo

    2014-01-01

    Although hyperlipidemia, high blood pressure, and diabetes increase the risk of arteriosclerosis, it is not clear whether hyperuricemia increases the risk of arteriosclerosis or not. We examined the effects of uric acid and curative drugs for hyperuricemia on atherosclerosis-susceptible C57BL/6J apolipoprotein E-deficient (apoE(-/-)) mice. Male apoE(-/-) mice (age: 6 weeks) were fed a normal diet (normal diet group) or a uric acid-enriched diet. Mice fed the uric acid-enriched diet were divided into three groups and administered a drinking vehicle (high uric acid diet group), allopurinol (20 mg·kg(-1)·d(-1)), or benzbromarone (20 mg·kg(-1)·d(-1)) for 10 weeks. Serum uric acid concentrations were higher in the high uric acid diet group than in the normal diet group, and concentrations in the allopurinol and benzbromarone groups were lower than in the high uric acid diet group. Serum total cholesterol and triglyceride levels were lower in the allopurinol group than in the high uric acid diet group. Oxidative stress was lower in the benzbromarone group than in the high uric acid diet group. Atherosclerotic lesion areas were smaller in the allopurinol and benzbromarone groups than in the high uric acid diet group. Thus, hyperuricemia may not be an independent risk factor for arteriosclerosis; however, the administration of allopurinol and benzbromarone prevented the development of atherosclerosis in apoE(-/-) mice fed a uric acid-enriched diet. The anti-atherosclerotic effect was in part due to lower total cholesterol and oxidative stress in the serum. Other possible mechanisms underlying this effect should be investigated.

  20. A new factor X defect (factor X Padua 3): a compound heterozygous between true deficiency (Gly(380)-->Arg) and an abnormality (Ser(334)-->Pro).

    Science.gov (United States)

    Vianello, F; Lombardi, A M; Boldrin, C; Luni, S; Girolami, A

    2001-11-15

    We report a novel mutation in Factor X (FX) gene which results in a phenotype without any bleeding tendency. The proband has been found to be a compound heterozygote between a novel FX true deficiency (Gly(380)-->Arg) and a previously reported dysfunctional mutation Ser(334)-->Pro (FX Marsiglia). Prothrombin time (PT) and partial thromboplastin time (PTT) were moderately prolonged and were fully corrected by the addition of normal serum. Her FX activity level varied between 8% and 19% of normal according to the method used whereas the FX antigen level was 40% of the normal control value. All the exons and intron/exon junctions of the FX gene were studied using a combined approach of polymerase chain reaction and conformation sensitive gel electrophoresis. A transversion G to A in exon 8 resulting in the replacement of Gly380 by Arg was found in the proband, in the father and in a proband's brother, whereas heterozygous FX Marsiglia was present in the proband's mother and her sister. Gly380 is strictly linked to Ser379, a component of the catalytic triad. The substitution of Gly for Arg causes the introduction of a large charged amino acid which could affect the catalytic function of FX leading to secretion problem, accounting for the cross-reactive material (CRM) negative phenotype.

  1. Iodine Deficiency

    Science.gov (United States)

    ... 2017 By ATA | Featured , Iodine Deficiency , News Releases , Potassium Iodide (KI) | No Comments IDD NEWSLETTER – February 2017 VOLUME ... 2016 By ATA | Featured , Iodine Deficiency , News Releases , Potassium Iodide (KI) | No Comments IDD NEWSLETTER – November 2015 (PDF ...

  2. Deficiency in the nuclear factor E2-related factor 2 renders pancreatic β-cells vulnerable to arsenic-induced cell damage

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Bei [Institute for Chemical Safety Sciences, The Hamner Institutes for Health Sciences, 6 Davis Drive, Research Triangle Park, NC 27709 (United States); Department of Histology and Embryology, College of Basic Medical Sciences, China Medical University, Shenyang 110001 (China); Fu, Jingqi; Zheng, Hongzhi; Xue, Peng; Yarborough, Kathy; Woods, Courtney G.; Hou, Yongyong; Zhang, Qiang; Andersen, Melvin E. [Institute for Chemical Safety Sciences, The Hamner Institutes for Health Sciences, 6 Davis Drive, Research Triangle Park, NC 27709 (United States); Pi, Jingbo, E-mail: jpi@thehamner.org [Institute for Chemical Safety Sciences, The Hamner Institutes for Health Sciences, 6 Davis Drive, Research Triangle Park, NC 27709 (United States)

    2012-11-01

    Chronic human exposure to inorganic arsenic (iAs), a potent environmental oxidative stressor, is associated with increased prevalence of type 2 diabetes, where impairment of pancreatic β-cell function is a key pathogenic factor. Nuclear factor E2-related factor 2 (Nrf2) is a central transcription factor regulating cellular adaptive response to oxidative stress. However, persistent activation of Nrf2 in response to chronic oxidative stress, including inorganic arsenite (iAs{sup 3+}) exposure, blunts glucose-triggered reactive oxygen species (ROS) signaling and impairs glucose-stimulated insulin secretion (GSIS). In the current study, we found that MIN6 pancreatic β-cells with stable knockdown of Nrf2 (Nrf2-KD) by lentiviral shRNA and pancreatic islets isolated from Nrf2-knockout (Nrf2−/−) mice exhibited reduced expression of several antioxidant and detoxification enzymes in response to acute iAs{sup 3+} exposure. As a result, Nrf2-KD MIN6 cells and Nrf2−/− islets were more susceptible to iAs{sup 3+} and monomethylarsonous acid (MMA{sup 3+})-induced cell damage, as measured by decreased cell viability, augmented apoptosis and morphological change. Pretreatment of MIN6 cells with Nrf2 activator tert-butylhydroquinone protected the cells from iAs{sup 3+}-induced cell damage in an Nrf2-dependent fashion. In contrast, antioxidant N‐acetyl cysteine protected Nrf2-KD MIN6 cells against acute cytotoxicity of iAs{sup 3+}. The present study demonstrates that Nrf2-mediated antioxidant response is critical in the pancreatic β-cell defense mechanism against acute cytotoxicity by arsenic. The findings here, combined with our previous results on the inhibitory effect of antioxidants on ROS signaling and GSIS, suggest that Nrf2 plays paradoxical roles in pancreatic β-cell dysfunction induced by environmental arsenic exposure. -- Highlights: ► Lack of Nrf2 reduced expression of antioxidant genes induced by iAs{sup 3+} in β-cells. ► Deficiency of Nrf2 in

  3. Adult-onset deficiency in growth hormone and insulin-like growth factor-I alters oligodendrocyte turnover in the corpus callosum.

    Science.gov (United States)

    Hua, Kun; Forbes, M Elizabeth; Lichtenwalner, Robin J; Sonntag, William E; Riddle, David R

    2009-08-01

    Growth hormone (GH) and insulin-like growth factor-I (IGF-I) provide trophic support during development and also appear to influence cell structure, function and replacement in the adult brain. Recent studies demonstrated effects of the GH/IGF-I axis on adult neurogenesis, but it is unclear whether the GH/IGF-I axis influences glial turnover in the normal adult brain. In the current study, we used a selective model of adult-onset GH and IGF-I deficiency to evaluate the role of GH and IGF-I in regulating glial proliferation and survival in the adult corpus callosum. GH/IGF-I-deficient dwarf rats of the Lewis strain were made GH/IGF-I replete via twice daily injections of GH starting at postnatal day 28 (P28), approximately the age at which GH pulse amplitude increases in developing rodents. GH/IGF-I deficiency was initiated in adulthood by removing animals from GH treatment. Quantitative analyses revealed that adult-onset GH/IGF-I deficiency decreased cell proliferation in the white matter and decreased the survival of newborn oligodendrocytes. These findings are consistent with the hypothesis that aging-related changes in the GH/IGF-I axis produce deficits in ongoing turnover of oligodendrocytes, which may contribute to aging-related cognitive changes and deficits in remyelination after injury.

  4. Revisiting the molecular epidemiology of factor XI deficiency: nine new mutations and an original large 4qTer deletion in western Brittany (France).

    Science.gov (United States)

    Guéguen, Paul; Chauvin, Angélique; Quémener-Redon, Sylvia; Pan-Petesch, Brigitte; Férec, Claude; Abgrall, Jean-François; Le Maréchal, Cédric

    2012-01-01

    Constitutional deficiency in factor XI (FXI) is a rare bleeding disorder in the general population, with the exception of Ashkenazi Jews. During the last decade, the detection of FXI-deficient patients has shifted from clinical screening identifying mostly severe bleeders to biological screening combining findings of prolonged activated partial thromboplastin time and FXI coagulation activity (FXI:C) below 50 U/dl. The goal of this study was to determine the molecular basis of FXI deficiency in western Brittany, France. Over the course of four years, we detected 98 FXI-deficient patients through biological screening, and 44 patients agreed to participate in this study corresponding to 25 index cases. We developed an efficient mutation detection strategy (combining direct sequencing and QFM-PCR to search for heterozygous rearrangements in a routine setting) that detected F11 mutations in 24 out of the 25 index cases. An unexpected allelic heterogeneity was found, with 14 different single point mutations being detected, among which nine are new. Moreover, a large heterozygous deletion of the entire F11 gene was detected, and was then further defined using a CGH array as a 4q34.2 telomeric deletion of 7 Mb containing 77 genes. We propose that the observed recurrent mutations may be considered as genetic tags of a population. This study highlights the importance of screening for large deletions in molecular studies of F11 .

  5. The predictive factors of low serum 25-hydroxyvitamin D and vitamin D deficiency in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Sumethkul, Kittiwan; Boonyaratavej, Smonporn; Kitumnuaypong, Tasanee; Angthararuk, Sungchai; Cheewasat, Patcharin; Manadee, Naruimon; Sumethkul, Vasant

    2013-06-01

    Vitamin D is a steroid hormone with pleiotropic effects. The association between serum 25-hydroxyvitamin D level [25(OH) D] and lupus nephritis are not clearly known. We aim to determine serum 25(OH) D levels in patients with inactive SLE, active SLE without lupus nephritis (LN) and active SLE with LN and to identify clinical predictor of vitamin D deficiency. One hundred and eight SLE patients were included. Patients were classified as Group (Gr) 1, 2 and 3 if they had SLE disease activity index (SLEDAI) vitamin D insufficiency and 29.6 % had vitamin D deficiency. Mean 25(OH) D in each groups was 28.3 (8.0), 26.7 (9.5) and 19.9 (7.6) ng/ml (p Vitamin D deficiency was found in 11.1, 22.2 and 55.6 % of Gr1, 2 and 3. Linear regression analysis found that 25(OH) D was significantly correlated with serum albumin (r = 0.28, p = 0.004), inversely correlated with SLEDAI (r = -0.22, p = 0.03) and urinary protein creatinine index (UPCI) (r = -0.28, p = 0.005), but not with sun exposure score, body mass index and estimated GFR. Only UPCI was significantly inversely correlated with 25(OH) D (p = 0.02) from multiple linear regression. LN was a significant predictor of vitamin D deficiency from multivariate logistic regression (OR 5.97; p = 0.006). Vitamin D deficiency and insufficiency was found in 93 and 86 % of LN with proteinuria ≥ and vitamin D level than inactive SLE and active SLE without LN. Hence, nephritis is a significant predictor of vitamin D deficiency in SLE patients.

  6. The ambiguity of ethnicity as risk factor of vitamin D deficiency - A case study of Danish vitamin D policy documents

    DEFF Research Database (Denmark)

    Mygind, Anna; Traulsen, Janine Morgall; Nørgaard, Lotte Stig;

    2011-01-01

    Objectives: To explore how ethnic minorities at risk of vitamin D deficiency are constructed in Danish policy documents (current as of April 2009), regarding vitamin D supplementation. Methods: Ten policy documents were analysed through content analysis, focusing on definitions and explanations...... are constructed very differently in Danish policies current as of April 2009. A more precise definition of ethnic minorities in policies and research may be helpful in seeking to identify which ethnic minorities are and are not at risk of vitamin D deficiency....

  7. Dirac R-matrix calculations of photoionization cross sections of Ni XII and atomic structure data of Ni XIII

    Science.gov (United States)

    Nazir, R. T.; Bari, M. A.; Bilal, M.; Sardar, S.; Nasim, M. H.; Salahuddin, M.

    2017-02-01

    We performed R-matrix calculations for photoionization cross sections of the two ground state configuration 3s23p5 (^2P^o3/2,1/2) levels and 12 excited states of Ni XII using relativistic Dirac Atomic R-matrix Codes (DARC) across the photon energy range between the ionizations thresholds of the corresponding states and well above the thresholds of the last level of the Ni XIII target ion. Generally, a good agreement is obtained between our results and the earlier theoretical photoionization cross sections. Moreover, we have used two independent fully relativistic GRASP and FAC codes to calculate fine-structure energy levels, wavelengths, oscillator strengths, transitions rates among the lowest 48 levels belonging to the configuration (3s23p4, 3s3p5, 3p6, 3s23p33d) in Ni XIII. Additionally, radiative lifetimes of all the excited states of Ni XIII are presented. Our results of the atomic structure of Ni XIII show good agreement with other theoretical and experimental results available in the literature. A good agreement is found between our calculated lifetimes and the experimental ones. Our present results are useful for plasma diagnostic of fusion and astrophysical plasmas.

  8. Proceedings of the XIII International Symposium on Biological Control of Weeds; September 11-16, 2011; Waikoloa, Hawaii, USA

    Science.gov (United States)

    Yun Wu; Tracy Johnson; Sharlene Sing; S. Raghu; Greg Wheeler; Paul Pratt; Keith Warner; Ted Center; John Goolsby; Richard Reardon

    2013-01-01

    A total of 208 participants from 78 organizations in 19 countries gathered at the Waikoloa Beach Marriott on the Big Island of Hawaii on September 11-16, 2011 for the XIII International Symposium on Biological Control of Weeds. Following a reception on the first evening, Symposium co-chairs Tracy Johnson and Pat Conant formally welcomed the attendees on the morning of...

  9. León XIII y la memoria de un catolicismo que «nació libre»

    Directory of Open Access Journals (Sweden)

    Robles Muñoz, Cristóbal

    2004-06-01

    Full Text Available Problems and responses during the pontificate of León XIII experienced changes of orientation after arrival of Pío XII. One of the problems was how to extend the presence of the Church among society, and expand relations of the latest with the Church. Responses were freedom and justice. Freedom to reflect on faith, thinking over its origins and dialoguing with culture, and to use recognised civil rights. Justice as a moral compromise towards the inhuman life conditions suffered by the population. León XIII was frightened at any innovation, but accepted the debate.

    Los problemas y respuestas presentes en la etapa de León XIII experimentaron un cambio de rumbo con la llegada de Pío X. Los problemas enraízan la presencia de la Iglesia en la sociedad y de ésta en la Iglesia. Las respuestas, la libertad y la justicia. La libertad, para pensar la fe, investigando sus orígenes y dialogando con la cultura, para usar derechos civilmente reconocidos. La justicia, como exigencia moral frente a las condiciones inhumanas en las que vive el pueblo. León XIII temió las novedades, pero aceptó el debate.

  10. THE CLINICAL EXPRESSION OF HEREDITARY PROTEIN-C AND PROTEIN-S DEFICIENCY - A RELATION TO CLINICAL THROMBOTIC RISK-FACTORS AND TO LEVELS OF PROTEIN-C AND PROTEIN-S

    NARCIS (Netherlands)

    HENKENS, CMA; VANDERMEER, J; HILLEGE, JL; BOM, VJJ; HALIE, MR; van der Schaaf, W

    1993-01-01

    We investigated 103 first-degree relatives of 13 unrelated protein C or protein S deficient patients to assess the role of additional thrombotic risk factors and of protein C and protein S levels in the clinical expression of hereditary protein C and protein S deficiency. Fifty-seven relatives were

  11. Vitamin A, E, and D Deficiencies in Tunisian Very Low Birth Weight Neonates: Prevalence and Risk Factors

    Directory of Open Access Journals (Sweden)

    Samira Fares

    2014-06-01

    Conclusion: Vitamin A, E, and D deficiencies are very common in Tunisian VLBW neonates and are associated with pre-eclampsia. Improved nutritional and health support for pregnant women and high dose vitamins A, E, and D supplementation in VLBW neonates are strongly required in Tunisia.

  12. Mice deficient for all PIM kinases display reduced body size and impaired responses to hematopoietic growth factors

    NARCIS (Netherlands)

    Mikkers, H; Nawijn, M; Allen, J; Brouwers, C; Verhoeven, E; Jonkers, J; Berns, A

    2004-01-01

    The Pim family of proto-oncogenes encodes a distinct class of serine/threonine kinases consisting of PIM1, PIM2, and PIM3. Although the Pim genes are evolutionarily highly conserved, the contribution of PIM proteins to mammalian development is unclear. PIM1-deficient mice were previously described b

  13. NCAM-mimetic, FGL peptide, restores disrupted fibroblast growth factor receptor (FGFR) phosphorylation and FGFR mediated signaling in neural cell adhesion molecule (NCAM)-deficient mice

    DEFF Research Database (Denmark)

    Aonurm-Helm, Anu; Berezin, Vladimir; Bock, Elisabeth;

    2010-01-01

    Neural cell adhesion molecule (NCAM) is a membrane-bound glycoprotein expressed on the surface of neuronal and glial cells. Previous in vitro studies have demonstrated that NCAM promotes neuronal functions largely via three main interaction partners: the fibroblast growth factor receptor (FGFR...... compared the levels of phosphorylation of FGFR1, Src kinase Fyn, Raf1 kinase, MAP kinases, Akt kinase and calcium/calmodulin-dependent kinases II and IV (CaMKII and CaMKIV) in the hippocampus of NCAM knockout mice to their wild-type littermates. The data of our study show that mice constitutively deficient...... in all isoforms of NCAM have decreased basal phosphorylation levels of FGFR1 and CaMKII and CaMKIV. Furthermore, NCAM-mimetic, FGL peptide, is found to be able to restore FGFR1, CaMKII and CaMKIV phosphorylation levels and thereby mimic the interactions of NCAM at this receptor in NCAM deficient mice...

  14. Decreased body fat, elevated plasma transforming growth factor-β levels, and impaired BMP4-like signaling in biglycan-deficient mice.

    Science.gov (United States)

    Tang, Tao; Thompson, Joel C; Wilson, Patricia G; Nelson, Christina; Williams, Kevin Jon; Tannock, Lisa R

    2013-01-01

    Biglycan (BGN), a small leucine-rich proteoglycan, binds the pro-fibrotic cytokine transforming growth factor β (TGFβ) and inhibits its bioactivity in vitro. Nevertheless, it is controversial whether BGN plays an inhibitory role in vivo. Therefore, the purpose of this study was to evaluate the effect of BGN deficiency on TGFβ activity in vivo by studying 1-year-old Bgn null and wild-type (WT) mice on an Ldlr-null background. Phenotypic and metabolic characterization showed that the Bgn null mice had lower body weight, shorter body length, and shorter femur length (all p kidney histology. Overall, we propose that this unexpected phenotype arises from the effects of BGN deficiency in vivo to elevate TGFβ levels while decreasing bone morphogenetic protein 4-like signaling.

  15. Fatores associados a anemia por deficiência de ferro em crianças pré-escolares brasileiras Factors associated with iron deficiency anemia in Brazilian preschool children

    Directory of Open Access Journals (Sweden)

    Carlos A. N. de Almeida

    2004-06-01

    Full Text Available OBJETIVO: Avaliar fatores determinantes de anemia e deficiência de ferro em crianças de duas creches da cidade de Pontal, sudeste do Brasil. MÉTODOS: Estudo transversal foi realizado avaliando-se 192 crianças com idades entre 12 e 72 meses. Dados pessoais (idade, sexo, uso de ferro medicamentoso, duração do aleitamento materno, tipo de parto, cuidados pré-natais, peso e estatura e dados socioeconômicos (número de co-habitantes, escolaridade dos pais e renda per capita familiar foram obtidos e correlacionados com hemoglobina, receptores de transferrina, ferritina e anemia ferropriva. RESULTADOS: A idade foi a variável mais afetada pelo estado nutricional de ferro, correlacionando-se com maiores valores de hemoglobina e ferritina e menores valores de receptor de transferrina, sendo que menos anemia ferropriva foi detectada quanto maior a idade. As outras variáveis estudadas não apresentaram correlação com o estado nutricional de ferro. CONCLUSÃO: Os dados sugerem que as estratégias de controle para essa população de crianças pré-escolares devem ser direcionadas especialmente para aquelas de menor idade.OBJECTIVE: To examine the determining factors of anemia and iron deficiency in children attending two day care centers in the town of Pontal, southeast of Brazil. METHODS: Cross-sectional study was conducted in 192 children aged 12 to 72 months. Personal data (age, sex, use of medicinal iron supplements, duration of breast-feeding, type of delivery, prenatal care, weight, and height, and socioeconomic data (number of co-inhabitants, parental schooling, and per capita family income were obtained and evaluated together with hemoglobin, serum transferrin receptor, ferritin, and iron deficiency anemia. RESULTS: Age was the variable that most affected iron nutritional status, with higher hemoglobin values, lower transferrin receptor concentrations, higher ferritin values and lower iron deficiency anemia being detected with increasing

  16. Vitamin D deficiency as a risk factor for the development of autoantibodies in patients with ASIA and silicone breast implants: a cohort study and review of the literature.

    Science.gov (United States)

    Colaris, Maartje J L; van der Hulst, Rene R; Tervaert, Jan Willem Cohen

    2017-03-17

    The development of autoimmunity and/or autoimmune diseases is multifactorial. Vitamin D is one of the factors that might play a role. We postulated that both the presence of adjuvants and insufficient levels of vitamin D may result in the development of autoimmunity in patients with autoimmune/inflammatory syndrome induced by adjuvants (ASIA) in relation to silicone implant incompatibility. We measured vitamin D levels in 135 patients with ASIA in relation to silicone implant incompatibility and related findings to the presence of autoantibodies that are commonly used to diagnose systemic autoimmune diseases. Furthermore, we systematically reviewed the literature regarding vitamin D deficiency as a risk factor for the development of autoantibodies. Vitamin D measurements were available for analysis in 131 of 135 patients with ASIA in relation to SIIS. Twenty-three patients (18%) tested positive for autoantibodies, from which 18 patients (78%) had either a vitamin D deficiency or insufficiency (median vitamin D level 60.5 mmol/L), whereas five patients (22%) had sufficient vitamin D levels. The risk to develop autoantibodies was significantly increased in vitamin D deficient and/or insufficient patients [RR 3.14; 95% CI, 1.24-7.95; p = 0.009]. Reviewed literature suggested an association between vitamin D levels and the presence and/or titer levels of autoantibodies in different autoimmune diseases. From our current study and from our review of the literature, we conclude that vitamin D deficiency is related to the presence of autoantibodies. Whether vitamin D supplementation results in a decrease of autoimmunity needs to be studied prospectively.

  17. Using a minigene approach to characterize a novel splice site mutation in human F7 gene causing inherited factor VII deficiency in a Chinese pedigree.

    Science.gov (United States)

    Yu, T; Wang, X; Ding, Q; Fu, Q; Dai, J; Lu, Y; Xi, X; Wang, H

    2009-11-01

    Factor VII deficiency which transmitted as an autosomal recessive disorder is a rare haemorrhagic condition. The aim of this study was to identify the molecular genetic defect and determine its functional consequences in a Chinese pedigree with FVII deficiency. The proband was diagnosed as inherited coagulation FVII deficiency by reduced plasma levels of FVII activity (4.4%) and antigen (38.5%). All nine exons and their flanking sequence of F7 gene were amplified by polymerase chain reaction (PCR) for the proband and the PCR products were directly sequenced. The compound heterozygous mutations of F7 (NM_000131.3) c.572-1G>A and F7 (NM_000131.3) c.1165T>G; p.Cys389Gly were identified in the proband's F7 gene. To investigate the splicing patterns associated with F7 c.572-1G>A, ectopic transcripts in leucocytes of the proband were analyzed. F7 minigenes, spanning from intron 4 to intron 7 and carrying either an A or a G at position -1 of intron 5, were constructed and transiently transfected into human embryonic kidney (HEK) 293T cells, followed by RT-PCR analysis. The aberrant transcripts from the F7 c.572-1G>A mutant allele were not detected by ectopic transcription study. Sequencing of the RT-PCR products from the mutant transfectant demonstrated the production of an erroneously spliced mRNA with exon 6 skipping, whereas a normal splicing occurred in the wide type transfectant. The aberrant mRNA produced from the F7 c.572-1G>A mutant allele is responsible for the factor VII deficiency in this pedigree.

  18. Disaccharidase deficiency.

    Science.gov (United States)

    Bayless, T M; Christopher, N L

    1969-02-01

    This review of the literature and current knowledge concerning a nutritional disorder of disaccharidase deficiency discusses the following topics: 1) a description of disorders of disaccharide digestion; 2) some historical perspective on the laboratory and bedside advances in the past 10 years that have helped define a group of these digestive disorders; 3) a classification of conditions causing disaccharide intolerance; and 4) a discussion of some of the specific clinical syndromes emphasizing nutritional consequences of these syndromes. The syndromes described include congenital lactase deficiency, acquired lactase deficiency in teenagers and adults, acquired generalized disaccharidase deficiency secondary to diffuse mucosal damage, acquired lactose intolerance secondary to alterations in the intestinal transit, sucrase-isomaltase deficiencies, and other disease associations connected with lactase deficiency such as colitis.

  19. Vitamin D deficiency in older adults and its associated factors: a cross-sectional analysis of the Mexican Health and Aging Study.

    Science.gov (United States)

    Carrillo-Vega, María Fernanda; García-Peña, Carmen; Gutiérrez-Robledo, Luis Miguel; Pérez-Zepeda, Mario Ulises

    2017-12-01

    Vitamin D deficiency was common in older adults from a country with adequate sun exposure. The variables associated with this deficiency provide insight into the next steps needed to characterize older adults with this deficiency and to treat it accordingly.

  20. Phylogenetic analysis of F-bZIP transcription factors indicates conservation of the zinc deficiency response across land plants

    DEFF Research Database (Denmark)

    Castro, Pedro Humberto Araújo R F; Lilay, Grmay Hailu; Muñoz-Mérida, Antonio

    2017-01-01

    data, we performed an evolutionary and structural characterization of plant F-bZIPs. We observed divergence during seed plant evolution, into two groups and inferred different selective pressures for each. Group 1 contains AtbZIP19 and AtbZIP23 and appears more conserved, whereas Group 2, containing...... be the result of neo-functionalization, the AtbZIP19/23 function in the regulation of the zinc deficiency response may be conserved in land plants (Embryophytes)....

  1. Predictors of Insulin Like Growth Factor-I responses to Growth Hormone replacement in young adults with Growth Hormone deficiency

    OpenAIRE

    Thankamony, Ajay; Capalbo, Donatella; Jonsson, Peter J.; Simpson, Helen L.; Dunger, David B.

    2016-01-01

    This is the author accepted manuscript. It is currently under an indefinite embargo pending publication by Karger Publishers. Background/Aims: Physiological growth hormone (GH) secretion and IGF-I levels are greater in young compared to older adults. We evaluated IGF-I levels and predictors of IGF-I responses in young adults on GH replacement. Design: From KIMS database, 310 young adults (age, 15 26 years) with the severe GH deficiency related to childhood-onset disease, and commenced ...

  2. PIXE and IL analysis of an archeologically problematic XIII century ceramic production

    Energy Technology Data Exchange (ETDEWEB)

    Zucchiatti, Alessandro, E-mail: alessandro.zucchiatti@uam.es [Centro de Micro-Análisis de Materiales, Universidad Autónoma de Madrid, C/Faraday 3, Madrid (Spain); Jiménez-Rey, David [Centro de Micro-Análisis de Materiales, Universidad Autónoma de Madrid, C/Faraday 3, Madrid (Spain); Laboratorio Nacional de Fusión CIEMAT, E06.P2.09, Madrid (Spain); Climent-Font, Aurelio [Centro de Micro-Análisis de Materiales, Universidad Autónoma de Madrid, C/Faraday 3, Madrid (Spain); Departamento di Física Aplicada, Universidad Autónoma de Madrid, Madrid (Spain); Martina, Silvia [Centro de Micro-Análisis de Materiales, Universidad Autónoma de Madrid, C/Faraday 3, Madrid (Spain); Universitá degli Studi di Torino, Turin (Italy); Faieta, Rosangela [Centro de Micro-Análisis de Materiales, Universidad Autónoma de Madrid, C/Faraday 3, Madrid (Spain); Universitá della Tuscia, Viterbo (Italy); Maggi, Marco [Musei Vaticani, Ufficio del Conservatore, Rome (Italy); Giuntini, Lorenzo; Calusi, Silvia [INFN – Laboratorio di tecniche Nucleari per i Beni Culturali, Florence (Italy)

    2015-11-15

    At the beginning of the XIII century the archaeologists have found evidence of a singular, transitional, pottery technique limited to a small area around western Liguria (Northwest of Italy). Known as Ligurian Protomajolica (PML), it shows in the same ceramic body and on the same surface white slip and enamel together, addressing questions about the technical reasons of this unusual combination, its origin and evolution. To integrate previous morphological and mineralogical studies, we have analysed by particle induced X-ray emission (also with mapping) and ionoluminescence (IL) the ceramic body, slip and glaze composition of 56 samples, of which 25 PML’s. We have identified some PML’s compositional features which are distinct from those of other coeval or later productions from the same area. A few PML imitations are described. A plausible explanation of the origin of the PML’s, based both on the archaeometric results and the archaeological and historical knowledge, is presented.

  3. Investidura de armas de los reyes españoles en los siglos XII y XIII

    Directory of Open Access Journals (Sweden)

    Palacios Martín, Bonifacio

    1988-12-01

    Full Text Available Este trabajo quiere ser una primera aproximación a uno de los temas más apasionantes para la simbologia política medieval: la investidura de armas del rey en la España cristiana de los siglos XII y XIII. El tema tiene todas las dificultades propias de los estudios de simbologia, que se acrecientan cuando se trata de simbologia política y más aún cuando se interfieren —como en este caso— conceptos caballerescos. Precisa entonces de unos planteamientos muy amplios que atiendan, por una parte, a la evolución pluriforme del acto de investidura, y por otra, al contexto sociopolitico en el que ésta se produce, a fin de captar los matices y circunstancias que inciden en su significado.

  4. Iron and FER-LIKE IRON DEFICIENCY-INDUCED TRANSCRIPTION FACTOR-dependent regulation of proteins and genes in Arabidopsis thaliana roots.

    Science.gov (United States)

    Mai, Hans-Jörg; Lindermayr, Christian; von Toerne, Christine; Fink-Straube, Claudia; Durner, Jörg; Bauer, Petra

    2015-09-01

    Iron is an essential micronutrient for plants, and iron deficiency requires a variety of physiological adaptations. FIT (FER-LIKE IRON DEFICIENCY-INDUCED TRANSCRIPTION FACTOR) is essential for the regulation of iron uptake in Arabidopsis thaliana roots. FIT is transcriptionally as well as posttranscriptionally regulated in response to iron supply. To investigate to which extent posttranscriptional regulation upon iron deficiency applies to proteins and to determine the dependency on FIT, we performed a parallel proteomic and transcriptomic study with wild-type, a fit knock-out mutant, and a FIT overexpressing Arabidopsis line. Among 92 proteins differentially regulated by iron and/or FIT, we identified 30 proteins, which displayed differential regulation at the transcriptional level. Eleven protein spots were regulated in at least one of the data points even contrary to the respective genes dependent on FIT. We found ten proteins in at least two forms. The analysis of functional classification showed enriched GO terms among the posttranscriptionally regulated genes and of proteins, that were downregulated or absent in the fit knock-out mutant. Taken together, we provide evidence for iron and FIT-dependent posttranscriptional regulation in iron homeostasis in A. thaliana.

  5. Relative Tissue Factor Deficiency Attenuates Ventilator-Induced Coagulopathy but Does Not Protect against Ventilator-Induced Lung Injury in Mice

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    Esther K. Wolthuis

    2012-01-01

    Full Text Available Preventing tissue-factor-(TF- mediated systemic coagulopathy improves outcome in models of sepsis. Preventing TF-mediated pulmonary coagulopathy could attenuate ventilator-induced lung injury (VILI. We investigated the effect of relative TF deficiency on pulmonary coagulopathy and inflammation in a murine model of VILI. Heterozygous TF knockout (TF+/− mice and their wild-type (TF+/+ littermates were sedated (controls or sedated, tracheotomized, and mechanically ventilated with either low or high tidal volumes for 5 hours. Mechanical ventilation resulted in pulmonary coagulopathy and inflammation, with more injury after mechanical ventilation with higher tidal volumes. Compared with TF+/+ mice, TF+/− mice demonstrated significantly lower pulmonary thrombin-antithrombin complex levels in both ventilation groups. There were, however, no differences in lung wet-to-dry ratio, BALF total protein levels, neutrophil influx, and lung histopathology scores between TF+/− and TF+/+ mice. Notably, pulmonary levels of cytokines were significantly higher in TF+/− as compared to TF+/+ mice. Systemic levels of cytokines were not altered by the relative absence of TF. TF deficiency is associated with decreased pulmonary coagulation independent of the ventilation strategy. However, relative TF deficiency does not reduce VILI and actually results in higher pulmonary levels of inflammatory mediators.

  6. Intestinal blood loss as an aggravating factor of iron deficiency in infants aged 9 to 12 months fed whole cow's milk.

    Science.gov (United States)

    Fernandes, Sandra Maria Rodrigues; de Morais, Mauro Batista; Amancio, Olga Maria Silverio

    2008-02-01

    To verify the occurrence of occult intestinal blood loss and iron deficiency in infants aged 9 to 12 months. A consecutive sample of 98 infants of the Pediatric Public Health Primary Care Unit in the town of Arapongas, Parana State, Brazil was involved in this cross-sectional study. Dietary history, hemoglobin, serum iron, transferrin saturation, ferritin, and an occult fecal blood loss investigation, by the immune chromatographic method specific for human hemoglobin were performed. Presence of occult intestinal blood occurred in 8/23 of the breast-fed (plus complementary feed) infants and in 30/64 of the infants who were fed with cow's milk (plus complementary feed) (P=0.449). The comparison of body iron indicators in accordance to positive or negative occult fecal blood, did not show any significant difference in the 23 breast-fed infants. Serum ferritin (median=4.2 ng/mL) was significantly lower (P=0.004) in infants who received whole cow's milk and had positive occult fecal blood, than in those infants who received whole cow's milk but were without occult fecal blood (median=12.1 ng/mL). In breast-fed infants with negative occult fecal blood, iron deficiency severity is not greater than in those with positive results. In infants fed whole cow's milk, occult fecal blood loss is an aggravating factor of iron deficiency.

  7. Characterisation of a large complex intragenic re-arrangement in the FVII gene (F7) avoiding misdiagnosis in inherited factor VII deficiency.

    Science.gov (United States)

    Giansily-Blaizot, Muriel; Thorel, Delphine; Khau Van Kien, Philippe; Behar, Catherine; Romey, Marie-Catherine; Mugneret, Francine; Schved, Jean-François; Claustres, Mireille

    2007-08-01

    Inherited factor VII (FVII) deficiency is a rare autosomal recessive bleeding disorder mostly caused by point mutations. Large genomic re-arrangements at F7 locus could account for a fraction of mutant alleles that remain unidentified after DNA sequencing, because they escape conventional polymerase chain reaction (PCR)-based techniques. We report the first systematic screening of F7 for large re-arrangements, by semi-quantitative multiplex PCR of fluorescent fragments targeting the 9 exons and the promoter region. A well-characterised cohort of 43 unrelated patients either apparently homozygous for a F7 point mutation or carrying at least one unidentified F7 mutant allele participated in this study. Two large F7 re-arrangements were identified in two FVII-deficient pedigrees, including a discontinuous deletion involving two distinct portions of F7 whose proximal and distal end junctions were characterised. A simple and efficient method for the routine detection of gross alterations of F7, which accounted for 2.3% of mutant alleles in our sample, is now available in inherited FVII deficiency. This test should complement conventional PCR-based techniques not only in unsolved cases, but also where inheritance pattern analysis is not achievable.

  8. [Iron deficiency, thrombocytosis and thromboembolism].

    Science.gov (United States)

    Evstatiev, Rayko

    2016-10-01

    Iron deficiency, the most common nutritional deficiency worldwide, is often associated with reactive thrombocytosis. Although secondary thrombocytosis is commonly considered to be harmless, there is accumulating evidence that elevated platelet counts, especially in the setting of iron deficiency, can lead to an increased thromboembolic risk in both arterial and venous systems. Here we present the mechanisms of iron deficiency-induced thrombocytosis and summarize its clinical consequences especially in patients with inflammatory bowel diseases, chronic kidney disease or cancer. We hypothesize that iron deficiency is an underestimated thromboembolic risk factor, and that iron replacement therapy can become an effective preventive strategy in a variety of clinical settings.

  9. Prolidase deficiency

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    Masood Qazi

    2007-01-01

    Full Text Available Prolidase deficiency is a rare inborn disorder of collagen metabolism characterized by chronic recurrent skin ulceration. A seven-year-old girl and her younger sibling with clinical features and laboratory criteria fulfilling the diagnosis of prolidase deficiency are presented in view of rarity of the condition.

  10. Iodine Deficiency

    NARCIS (Netherlands)

    Zimmermann, M.B.

    2009-01-01

    Iodine deficiency has multiple adverse effects in humans, termed iodine deficiency disorders, due to inadequate thyroid hormone production. Globally, it is estimated that 2 billion individuals have an insufficient iodine intake, and South Asia and sub-Saharan Africa are particularly affected. Howeve

  11. Iodine Deficiency

    NARCIS (Netherlands)

    Zimmermann, M.B.

    2009-01-01

    Iodine deficiency has multiple adverse effects in humans, termed iodine deficiency disorders, due to inadequate thyroid hormone production. Globally, it is estimated that 2 billion individuals have an insufficient iodine intake, and South Asia and sub-Saharan Africa are particularly affected. Howeve

  12. Related Factors of Pregnant Women with Iron Deficiency Anemia in 1168 Cases%1168例孕妇缺铁性贫血影响因素分析

    Institute of Scientific and Technical Information of China (English)

    姜波玲; 卢媛

    2015-01-01

    Objective To analyze the related factors of pregnant women with iron deficiency anemia, and provide reliable basis for health care during pregnancy. Methods The pregnant women building the perinatal care Handbook in maternal and child health care center of Wuchang district, divided into two groups of iron deficiency anemia group of 342 cases and a control group of 826 cases according to thehemoglobin and serum fer itin were carried on the questionnaire survey and dietary analysis, and final y a chi square test was used for data analysis. Results Iron deficiency anemia in pregnancy occur ed in 29.3%, there were significant dif erences in age, cultural level, family income, body mass index, the pregnancy reaction,dietary iron intake of two pregnant women ( <0.01).Conclusion Age, cultural level, family income, body mass index, early pregnancy reaction, dietary iron intake and iron deficiency anemia in pregnancy is related closely. Inadequate dietary iron intake during pregnancy is an important influence factor of iron deficiency anemia during pregnancy.%目的探讨孕妇孕期缺铁性贫血的相关影响因素,为孕期保健提供可靠的依据。方法对来武昌区妇幼保健中心建围产保健手册的孕妇,按血色素和血清铁蛋白分为缺铁性贫血组342例和对照组826例进行问卷调查及膳食分析,最后采用字2检验进行数据分析。结果妊娠期缺铁性贫血发生率29.3%,两组孕妇在年龄、文化程度、家庭收入、体重指数、早孕反应、膳食铁摄入六个方面存在统计学差异(<0.01)。结论年龄、文化程度、家庭收入、体重指数、早孕反应、膳食铁摄入与妊娠期缺铁性贫血密切相关,孕期膳食铁摄入不合理是妊娠期缺铁性贫血的重要影响因素。

  13. Heerlen polymorphism associated with type III protein S deficiency and factor V Leiden mutation in a Polish patient with deep vein thrombosis.

    Science.gov (United States)

    Wypasek, Ewa; Potaczek, Daniel P; Alhenc-Gelas, Martine; Undas, Anetta

    2014-01-01

    Protein S is one of the major natural anticoagulants. A missense serine 501 to proline (S501P) Heerlen polymorphism is associated with reduced levels of free protein S. Heerlen polymorphism, especially when combined with other thrombophilia risk factors, can lead to thromboembolic complications. To our knowledge, we report here the first Polish case associated with heterozygous Heerlen polymorphism resulting in type III protein S deficiency, detected in a 50-year-old man with several thrombotic episodes of deep and superficial veins and a highly positive thrombotic family history. The patient also had factor V Leiden mutation and persistently elevated anticardiolipin antibodies. It seems that increased risk of thrombotic complications could be explained in the patient by a synergy between the effects of Heerlen polymorphism, factor V Leiden heterozygous status and antiphospholipid syndrome.

  14. Hepatic deficiency of the pioneer transcription factor FoxA restricts hepatitis B virus biosynthesis by the developmental regulation of viral DNA methylation.

    Directory of Open Access Journals (Sweden)

    Vanessa C McFadden

    2017-02-01

    Full Text Available The FoxA family of pioneer transcription factors regulates hepatitis B virus (HBV transcription, and hence viral replication. Hepatocyte-specific FoxA-deficiency in the HBV transgenic mouse model of chronic infection prevents the transcription of the viral DNA genome as a result of the failure of the developmentally controlled conversion of 5-methylcytosine residues to cytosine during postnatal hepatic maturation. These observations suggest that pioneer transcription factors such as FoxA, which mark genes for expression at subsequent developmental steps in the cellular differentiation program, mediate their effects by reversing the DNA methylation status of their target genes to permit their ensuing expression when the appropriate tissue-specific transcription factor combinations arise during development. Furthermore, as the FoxA-deficient HBV transgenic mice are viable, the specific developmental timing, abundance and isoform type of pioneer factor expression must permit all essential liver gene expression to occur at a level sufficient to support adequate liver function. This implies that pioneer transcription factors can recognize and mark their target genes in distinct developmental manners dependent upon, at least in part, the concentration and affinity of FoxA for its binding sites within enhancer and promoter regulatory sequence elements. This selective marking of cellular genes for expression by the FoxA pioneer factor compared to HBV may offer the opportunity for the specific silencing of HBV gene expression and hence the resolution of chronic HBV infections which are responsible for approximately one million deaths worldwide annually due to liver cirrhosis and hepatocellular carcinoma.

  15. Association between anaemia, iron deficiency anaemia, neglected parasitic infections and socioeconomic factors in rural children of West Malaysia.

    Directory of Open Access Journals (Sweden)

    Romano Ngui

    Full Text Available BACKGROUND: Given that micronutrient deficiency, neglected intestinal parasitic infections (IPIs and poor socioeconomic status are closely linked, we conducted a cross-sectional study to assess the relationship between IPIs and nutritional status of children living in remote and rural areas in West Malaysia. METHODS/FINDINGS: A total of 550 children participated, comprising 520 (94.5% school children aged 7 to 12 years old, 30 (5.5% young children aged 1 to 6 years old, 254 (46.2% boys and 296 (53.8% girls. Of the 550 children, 26.2% were anaemic, 54.9% iron deficient and 16.9% had iron deficiency anaemia (IDA. The overall prevalence of helminths was 76.5% comprising Trichuris trichiura (71.5%, Ascaris lumbricoides (41.6% and hookworm infection (13.5%. It was observed that iron deficiency was significantly higher in girls (p = 0.032 compared to boys. Univariate analysis demonstrated that low level of mother's education (OR = 2.52; 95% CI = 1.38-4.60; p = 0.002, non working parents (OR = 2.18; 95% CI = 2.06-2.31; p = 0.013, low household income (OR = 2.02; 95% CI = 1.14-3.59; p = 0.015, T. trichiura (OR = 2.15; 95% CI = 1.21-3.81; p = 0.008 and A. lumbricoides infections (OR = 1.63; 95% CI = 1.04-2.55; p = 0.032 were significantly associated with the high prevalence of IDA. Multivariate analysis confirmed that low level of mother's education (OR = 1.48; 95 CI% = 1.33-2.58; p<0.001 was a significant predictor for IDA in these children. CONCLUSION: It is crucial that a comprehensive primary health care programme for these communities that includes periodic de-worming, nutrition supplement, improved household economy, education, sanitation status and personal hygiene are taken into consideration to improve the nutritional status of these children.

  16. Association between Anaemia, Iron Deficiency Anaemia, Neglected Parasitic Infections and Socioeconomic Factors in Rural Children of West Malaysia

    Science.gov (United States)

    Ngui, Romano; Lim, Yvonne Ai Lian; Chong Kin, Liam; Sek Chuen, Chow; Jaffar, Shukri

    2012-01-01

    Background Given that micronutrient deficiency, neglected intestinal parasitic infections (IPIs) and poor socioeconomic status are closely linked, we conducted a cross-sectional study to assess the relationship between IPIs and nutritional status of children living in remote and rural areas in West Malaysia. Methods/Findings A total of 550 children participated, comprising 520 (94.5%) school children aged 7 to 12 years old, 30 (5.5%) young children aged 1 to 6 years old, 254 (46.2%) boys and 296 (53.8%) girls. Of the 550 children, 26.2% were anaemic, 54.9% iron deficient and 16.9% had iron deficiency anaemia (IDA). The overall prevalence of helminths was 76.5% comprising Trichuris trichiura (71.5%), Ascaris lumbricoides (41.6%) and hookworm infection (13.5%). It was observed that iron deficiency was significantly higher in girls (p = 0.032) compared to boys. Univariate analysis demonstrated that low level of mother's education (OR = 2.52; 95% CI = 1.38–4.60; p = 0.002), non working parents (OR = 2.18; 95% CI = 2.06–2.31; p = 0.013), low household income (OR = 2.02; 95% CI = 1.14–3.59; p = 0.015), T. trichiura (OR = 2.15; 95% CI = 1.21–3.81; p = 0.008) and A. lumbricoides infections (OR = 1.63; 95% CI = 1.04–2.55; p = 0.032) were significantly associated with the high prevalence of IDA. Multivariate analysis confirmed that low level of mother's education (OR = 1.48; 95 CI% = 1.33–2.58; pchildren. Conclusion It is crucial that a comprehensive primary health care programme for these communities that includes periodic de-worming, nutrition supplement, improved household economy, education, sanitation status and personal hygiene are taken into consideration to improve the nutritional status of these children. PMID:22413027

  17. Vitamin B12 deficiency.

    Science.gov (United States)

    Oh, Robert; Brown, David L

    2003-03-01

    Vitamin B12 (cobalamin) deficiency is a common cause of macrocytic anemia and has been implicated in a spectrum of neuropsychiatric disorders. The role of B12 deficiency in hyperhomocysteinemia and the promotion of atherosclerosis is only now being explored. Diagnosis of vitamin B12 deficiency is typically based on measurement of serum vitamin B12 levels; however, about 50 percent of patients with subclinical disease have normal B12 levels. A more sensitive method of screening for vitamin B12 deficiency is measurement of serum methylmalonic acid and homocysteine levels, which are increased early in vitamin B12 deficiency. Use of the Schilling test for detection of pernicious anemia has been supplanted for the most part by serologic testing for parietal cell and intrinsic factor antibodies. Contrary to prevailing medical practice, studies show that supplementation with oral vitamin B12 is a safe and effective treatment for the B12 deficiency state. Even when intrinsic factor is not present to aid in the absorption of vitamin B12 (pernicious anemia) or in other diseases that affect the usual absorption sites in the terminal ileum, oral therapy remains effective.

  18. Identification of a new Newcastle disease virus isolate from Indonesia represents an ancestral lineage of class II genotype XIII.

    Science.gov (United States)

    Forrester, Naomi L; Widen, Steve G; Wood, Thomas G; Travassos da Rosa, Amelia P; Ksiazek, Thomas G; Vasilakis, Nikos; Tesh, Robert B

    2013-08-01

    An unknown virus was isolated from a mosquito pool collected in Jakarta during routine surveillance in 1979. Analysis of the sample using the Illumina platform resulted in the identification of a Newcastle disease virus (NDV) isolate. The sequence of the isolate indicated that it is an ancestral lineage of class II, genotype XIII. The source of the isolate is unusual, as newcastle disease virus is not believed to be vector-borne, although this mosquito pool was processed in a laboratory also handling samples for avian influenza surveillance and it is possible that this resulted in cross-contamination. This NDV isolate is still ancestral to most extant genotype XIII strains and provides a useful insight into historic NDV evolution.

  19. Who was the Red Queen? Identity of the female Maya dignitary from the sarcophagus tomb of Temple XIII, Palenque, Mexico.

    Science.gov (United States)

    Tiesler, V; Cucina, A; Pacheco, A Romano

    2004-01-01

    The present investigation aims at contributing to the ongoing discussion on the unconfirmed identity of the Red Queen, a Classic Maya dignitary discovered in Temple XIII at Palenque, Mexico, by comparing her reconstructed facial profile to the portraiture of known female personages from the site. The comparison rests upon individual cranial features, like buccal prognatism, nasal root and inclination, chin prominence and the artificially shaped forehead. The similarities between the reconstruction, the female's funerary mask and local portraiture appear to identify the Red Queen as Lady Ix Tz'akb'u Ajaw (Ahpo Hel), the wife of Janaab' Pakal, one of the famous Maya rulers of the Classic Period. The proposed match and her family relationship with the king might explain the spatial closeness of their burial places in the Temple of the Inscriptions and Temple XIII.

  20. Feeding practices and factors contributing to wasting, stunting, and iron-deficiency anaemia among 3-23-month old children in Kilosa district, rural Tanzania.

    Science.gov (United States)

    Mamiro, Peter S; Kolsteren, Patrick; Roberfroid, Dominique; Tatala, Simon; Opsomer, Ann S; Van Camp, John H

    2005-09-01

    Infants in Tanzania are particularly vulnerable to under-nutrition during transition from breastmilk (as the only source of nourishment) to solid foods. A cross-sectional study was undertaken in Kilosa district in Tanzania to determine the feeding practices and the extent of wasting, stunting, and iron-deficiency anaemia. The study was done in two stages: in the first stage, a 24-hour dietary assessment was conducted to identify the type of complementary foods given and the eating habits according to age for 378 children aged 3-23 months. In the second stage, a progressive recruitment of 309 infants aged six months was made to measure weight, length, haemoglobin (Hb) concentration, zinc protoporphyrin concentration, and malaria parasitaemia. Birth-weight, the potential contributing factor to under-nutrition and iron-deficiency anaemia, was obtained from the children's clinic cards. The 24-hour dietary assessment revealed that children consumed mainly a thin porridge prepared from maize flour as complementary food. Carbohydrates contributed most energy (on average 69%), followed by fats (18.6%) and protein (on average 12.1%). The complementary food co-vered only 15%, 20%, and 27% of the recommended iron intake for children aged 6-8, 9-11 and 12-23 months respectively. The mean Hb concentration was 9.3 +/- 1.9 g/dL, 68% of the infants were moderately anaemic (7 or =11 g/dL). Equally, the mean zinc protoporphyrin concentration was 10.0 +/- 6.2 microg/g Hb, and 76% of the infants were iron-deficient (>5 microg/g Hb). The prevalence of stunting was 35%, while wasting was only 1.3%. Low birth-weight and low body mass index of mothers were the strong predictors of stunting, whereas low birth-weight and iron-deficiency were the strong predictors of anaemia. The prevalence of malaria parasitaemia was high, affecting 50% of the infants. Having malaria was the only independent predictor associated with stunting, anaemia, and iron-deficiency. There is an urgent need to improve

  1. I governi di “popolo” e le istituzioni comunali nella seconda metà del secolo XIII

    Directory of Open Access Journals (Sweden)

    Enrico Artifoni

    2003-12-01

    Full Text Available Nella seconda metà del secolo XIII la cultura politica dei governi popolari si esprime in modo articolato e consapevole attraverso molteplici scelte: l’istituzione di organismi collegiali ristretti, la valorizzazione delle forme associative di quartiere o di rione, la “domanda” rivolta al ceto notarile di elaborare forme documentarie adeguate a un assetto istituzionale complesso qual era quello del comune duecentesco.

  2. Adipocyte-Specific Hypoxia-Inducible FactorDeficiency Exacerbates Obesity-Induced Brown Adipose Tissue Dysfunction and Metabolic Dysregulation.

    Science.gov (United States)

    García-Martín, Rubén; Alexaki, Vasileia I; Qin, Nan; Rubín de Celis, María F; Economopoulou, Matina; Ziogas, Athanasios; Gercken, Bettina; Kotlabova, Klara; Phieler, Julia; Ehrhart-Bornstein, Monika; Bornstein, Stefan R; Eisenhofer, Graeme; Breier, Georg; Blüher, Matthias; Hampe, Jochen; El-Armouche, Ali; Chatzigeorgiou, Antonios; Chung, Kyoung-Jin; Chavakis, Triantafyllos

    2015-11-16

    Angiogenesis is a central regulator for white (WAT) and brown (BAT) adipose tissue adaptation in the course of obesity. Here we show that deletion of hypoxia-inducible factor 2α (HIF2α) in adipocytes (by using Fabp4-Cre transgenic mice) but not in myeloid or endothelial cells negatively impacted WAT angiogenesis and promoted WAT inflammation, WAT dysfunction, hepatosteatosis, and systemic insulin resistance in obesity. Importantly, adipocyte HIF2α regulated vascular endothelial growth factor (VEGF) expression and angiogenesis of obese BAT as well as its thermogenic function. Consistently, obese adipocyte-specific HIF2α-deficient mice displayed BAT dysregulation, associated with reduced levels of uncoupling protein 1 (UCP1) and a dysfunctional thermogenic response to cold exposure. VEGF administration reversed WAT and BAT inflammation and BAT dysfunction in adipocyte HIF2α-deficient mice. Together, our findings show that adipocyte HIF2α is protective against maladaptation to obesity and metabolic dysregulation by promoting angiogenesis in both WAT and BAT and by counteracting obesity-mediated BAT dysfunction. Copyright © 2016 García-Martín et al.

  3. Prevalence of Factor V Leiden-G1691A and MTHFR-C677T Thrombosis Gene Modifier in Iron Deficiency Anemia: A Pathophysiological Effect in Indian Isolates.

    Science.gov (United States)

    Pandey, S K; Pandey, S; Mishra, R M; Indurkar, M

    2017-03-01

    Normal iron levels are required to prevent thrombocytosis by inhibiting thrombopoiesis. Thrombocytosis is usually associated with a mild iron deficiency and is the result of a lack of inhibition of thrombopoiesis. Study participants were 430 iron deficiency anemia (IDA) patients. Ten (10) mL of venous blood were collected for the subjects. Ferritin analysis was done by ELISA method while Hemogram analysis was done by auto-analyzer. Factor V Leiden, PRTG20210A, and MTHFR C677T genotype analysis was performed by PCR-RFLP method. Among the patients, 9 were heterozygous (G>A) and 2 were homozygous (A>A) carrier of FV Leiden; while 20 were heterozygous (C>T) and 3 were homozygous (T>T) for MTHFR polymorphism. None of the patient was identified with PT mutation. Patients with thrombosis gene marker had lower hemoglobin, mean corpuscular volume, mean corpuscular haemoglobin levels, and mean corpuscular hemoglobin concentration than patients without thrombosis gene marker. Serum ferritin was elevated in subject with the absence of thrombosis gene markers. Our data suggest a high impact of inherited hypercoagulability risk factors in the pathogenesis of IDA and its complications.

  4. Iodine Deficiency

    Science.gov (United States)

    ... 0 Iodine Daily Serving now recommended in Multivitamin/Mineral Supplements for Pregnant and Lactating Women By ATA | 2015 News Releases , Iodine Deficiency , News Releases , Thyroid Disease and Pregnancy | No Comments Falls Church, February 10, 2015 —The ...

  5. Iron deficiency.

    Science.gov (United States)

    Scrimshaw, N S

    1991-10-01

    The world's leading nutritional problem is iron deficiency. 66% of children and women aged 15-44 years in developing countries have it. Further, 10-20% of women of childbearing age in developed countries are anemic. Iron deficiency is identified with often irreversible impairment of a child's learning ability. It is also associated with low capacity for adults to work which reduces productivity. In addition, it impairs the immune system which reduces the body's ability to fight infection. Iron deficiency also lowers the metabolic rate and the body temperature when exposed to cold. Hemoglobin contains nearly 73% of the body's iron. This iron is always being recycled as more red blood cells are made. The rest of the needed iron does important tasks for the body, such as binds to molecules that are reservoirs of oxygen for muscle cells. This iron comes from our diet, especially meat. Even though some plants, such as spinach, are high in iron, the body can only absorb 1.4-7% of the iron in plants whereas it can absorb 20% of the iron in red meat. In many developing countries, the common vegetarian diets contribute to high rates of iron deficiency. Parasitic diseases and abnormal uterine bleeding also promote iron deficiency. Iron therapy in anemic children can often, but not always, improve behavior and cognitive performance. Iron deficiency during pregnancy often contributes to maternal and perinatal mortality. Yet treatment, if given to a child in time, can lead to normal growth and hinder infections. However, excess iron can be damaging. Too much supplemental iron in a malnourished child promotes fatal infections since the excess iron is available for the pathogens use. Many countries do not have an effective system for diagnosing, treating, and preventing iron deficiency. Therefore a concerted international effort is needed to eliminate iron deficiency in the world.

  6. Solubilization of immune complexes in complement factor deficient sera and the influence of temperature, ionic strength and divalent cations on the solubilization reaction

    DEFF Research Database (Denmark)

    Baatrup, Gunnar; Petersen, Ivan; Svehag, Svend-Erik;

    1984-01-01

    The complement-mediated solubilization (CMS) of immune complexes (IC) and the initial kinetics (IKS) of this reaction in human sera depleted of or deficient in C2, C3, C8, factors B, P and I were investigated. Sera depleted of B or P and those lacking native C3 or factor I showed virtually no CMS...... by a radioassay and kinetic data for the binding of C3b to preformed immune complexes. The CMS capacity reached maximum at 39-41 degrees C and at an ionic strength of approximately 0.20 mu. Selective chelation of Mg2+ completely abolished the CMS of IC. Maximal CMS was observed at Mg2+ concentration of about 2m...

  7. Del "scriptor" al "publicus notarius" : los escribanos de Madrid en el siglo XIII

    Directory of Open Access Journals (Sweden)

    María Teresa Carrasco Lazareno

    2003-01-01

    Full Text Available Estudio diplomático y jurídicoinstitucional de las escribanías de Madrid en el siglo XIII. Se analiza su evolución desde los primeros "Scriptores» prenotariales conocidos, clérigos y laicos de libre profesión, documentados hasta 1260, hasta la consolidación definitiva de la institución notarial y del instrumento público en el último cuarto del siglo XIII. Se estudian con detenimiento la compleja etapa de transición, en las décadas centrales de la centuria, la figura del «escrivano de congejo de Madrit», profesional estable y antecedente inmediato del escribano público, y la instauración del notariado, de acuerdo con la legislación alfonsina, así como de la escribanía municipal, por otorgamiento de Alfonso X, en 1264. A través de la documentación conservada, se analiza, asimismo, la transformación de la simple carta o «scriptura» testifical en «publicum instrumentum», mediante la valoración de la pervivencia de elementos tradicionales y la aparición de fórmulas diplomáticas que revelan la paulatina introducción.This is a diplomatic and a legal — institutional study about the actuarles offices in Madrid during the Xlllth century Its evolution is analyzed from the first known prenotarial "scriptores», priests and liberal professionals seculars, documented until 1260, until the final consolidation of the notary institution and the public instrument during the last quarter of the Xlllth century There is a detailed study about the complexity of the transitional period, during the central decades of that century, the figure of the «Notary of the Madrid Town Council», as a stable professional and immediate predecessor of the public actuary the establishment of the body of actuarles, according to the Alphonsine legislation, an the municipal actuary office, established by King Alphonse Xth., in 1264. Through the preserved documentation, it is analized, at the same time, the transformation of the simple letter, or

  8. Hyperplasia and hypertrophy of pulmonary neuroepithelial bodies, presumed airway hypoxia sensors, in hypoxia-inducible factor prolyl hydroxylase-deficient mice.

    Science.gov (United States)

    Pan, Jie; Bishop, Tammie; Ratcliffe, Peter J; Yeger, Herman; Cutz, Ernest

    2016-01-01

    Pulmonary neuroepithelial bodies (NEBs), presumed polymodal airway sensors, consist of innervated clusters of amine (serotonin) and peptide-producing cells. While NEB responses to acute hypoxia are mediated by a membrane-bound O2 sensor complex, responses to sustained and/or chronic hypoxia involve a prolyl hydroxylase (PHD)-hypoxia-inducible factor-dependent mechanism. We have previously reported hyperplasia of NEBs in the lungs of Phd1-/- mice associated with enhanced serotonin secretion. Here we use a novel multilabel immunofluorescence method to assess NEB distribution, frequency, and size, together with the number and size of NEB cell nuclei, and to colocalize multiple cytoplasmic and nuclear epitopes in the lungs of Phd1-/-, Phd2+/-, and Phd3-/- mice and compare them with wild-type controls. To define the mechanisms of NEB cell hyperplasia, we used antibodies against Mash1 and Prox1 (neurogenic genes involved in NEB cell differentiation/maturation), hypoxia-inducible factor-1alpha, and the cell proliferation marker Ki67. Morphometric analysis of (% total lung area) immunostaining for synaptophysin (% synaptophysin), a cytoplasmic marker of NEB cells, was significantly increased in Phd1-/- and Phd3-/- mice compared to wild-type mice. In addition, NEB size and the number and size of NEB nuclei were also significantly increased, indicating that deficiency of Phds is associated with striking hyperplasia and hypertrophy of NEBs. In Phd2+/- mice, while mean % synaptophysin was comparable to wild-type controls, the NEB size was moderately increased, suggesting an effect even in heterozygotes. NEBs in all Phd-deficient mice showed increased expression of Mash1, Prox1, Ki67, and hypoxia-inducible factor-1alpha, in keeping with enhanced differentiation from precursor cells and a minor component of cell proliferation. Since the loss of PHD activity mimics chronic hypoxia, our data provide critical information on the potential role of PHDs in the pathobiology and

  9. The effect of factor VIII deficiencies and replacement and bypass therapies on thrombus formation under venous flow conditions in microfluidic and computational models.

    Directory of Open Access Journals (Sweden)

    Abimbola A Onasoga-Jarvis

    Full Text Available Clinical evidence suggests that individuals with factor VIII (FVIII deficiency (hemophilia A are protected against venous thrombosis, but treatment with recombinant proteins can increase their risk for thrombosis. In this study we examined the dynamics of thrombus formation in individuals with hemophilia A and their response to replacement and bypass therapies under venous flow conditions. Fibrin and platelet accumulation were measured in microfluidic flow assays on a TF-rich surface at a shear rate of 100 s⁻¹. Thrombin generation was calculated with a computational spatial-temporal model of thrombus formation. Mild FVIII deficiencies (5-30% normal levels could support fibrin fiber formation, while severe (1 nM, but too low to support fibrin formation (<10 nM. In the absence of platelets, fibrin formation was not supported even at normal FVIII levels, suggesting platelet adhesion is necessary for fibrin formation. Individuals treated by replacement therapy, recombinant FVIII, showed normalized fibrin formation. Individuals treated with bypass therapy, recombinant FVIIa, had a reduced lag time in fibrin formation, as well as elevated fibrin accumulation compared to healthy controls. Treatment of rFVIIa, but not rFVIII, resulted in significant changes in fibrin dynamics that could lead to a prothrombotic state.

  10. Muscle force and endurance in untreated and human growth hormone or insulin-like growth factor-I-treated patients with growth hormone deficiency or Laron syndrome.

    Science.gov (United States)

    Brat, O; Ziv, I; Klinger, B; Avraham, M; Laron, Z

    1997-01-01

    Muscle force and endurance of four muscle groups (biceps, triceps, hamstrings and quadriceps) were measured by a computerized device in three groups: (A) 4 boys with isolated growth hormone deficiencies (IGHD) examined before at 10 and 24 months of hGH treatment; (B) 5 children (2 F, 3 M) with Laron syndrome were examined 3.5-4 years after initiation of insulin-like growth factor-I (IGF-I) treatment, and (C) comprised 8 untreated adults (5 F, 3 M) with Laron syndrome. For each patient, 2 matched controls, by age, sex, physical activity and height below the 50th percentile, were examined. GH- or IGF-I-deficient patients before treatment revealed reduced muscle force and endurance. GH treatment (0.6 U/kg/week) restored muscle force and endurance, progressively, mainly in the boys with puberty. Three to 4 years of IGF-I treatment (150 micrograms/kg/day) in patients with Laron syndrome proved to have a weaker effect than GH in restoring muscle force. The difference in effectiveness between hGH and IGF-I in restoring muscle force may be due to either the more marked muscle underdevelopment in Laron syndrome patients than in patients with IGHD or a difference in action potential between the two hormones.

  11. F7 gene variants modulate protein levels in a large cohort of patients with factor VII deficiency. Results from a genotype-phenotype study.

    Science.gov (United States)

    Quintavalle, Gabriele; Riccardi, Federica; Rivolta, Gianna Franca; Martorana, Davide; Di Perna, Caterina; Percesepe, Antonio; Tagliaferri, Annarita

    2017-08-01

    Congenital factor VII (FVII) deficiency is a rare bleeding disorder caused by mutations in F7 gene with autosomal recessive inheritance. A clinical heterogeneity with poor correlation with FVII:C levels has been described. It was the objective of this study to identify genetic defects and to evaluate their relationships with phenotype in a large cohort of patients with FVII:CF7 mutations and three polymorphic variants and classified according to recently published clinical scores. Forty out of 123 patients (33 %) were symptomatic (43 bleedings). A severe bleeding tendency was observed only in patients with FVII:CF7 international databases. Most mutations (62 %) were missense, large deletions were 6.2 %. Compound heterozygotes/homozygotes for mutations presented lower FVII:C levels compared to the other classes (Chi(2)=43.709, pF7 genotypic groups (Chi(2)=72.289, pF7 mutation and/or polymorphisms and FVII:C levels, without a direct link between FVII:C and bleeding tendency. The results suggest that large deletions are underestimated and that they represent a common mechanism of F7 gene inactivation which should always be investigated in the diagnostic testing for FVII deficiency.

  12. Prevalence of Vitamin D deficiency and associated risk factors among children residing at high altitude in Shimla district, Himachal Pradesh, India

    Science.gov (United States)

    Kapil, Umesh; Pandey, Ravindra Mohan; Goswami, Ravinder; Sharma, Brij; Sharma, Neetu; Ramakrishnan, Lakshmy; Singh, Gajendra; Sareen, Neha; Sati, Hem Chandra; Gupta, Aakriti; Sofi, Nighat Yaseen

    2017-01-01

    Introduction: Vitamin D is important for bone development in children. A high prevalence of Vitamin D deficiency (VDD) has been documented from different parts of India. However, limited data are available on VDD among children residing at high altitude region of country. Objectives: To assess the prevalence of VDD and associated risk factors among children in the age group of 6–18 years in Shimla, Himachal Pradesh. Methods: A community-based cross-sectional study was conducted in the year 2014–2015. A total of 626 children in the age group of 6–18 years were enrolled from 30 clusters which were identified using population proportionate to size sampling method. A minimum of 20 children in the age group of 6–18 years per cluster were selected using random number tables. The data on socioeconomic status, physical activity, sunlight exposure, and biochemical parameters of bone and mineral metabolism were assessed. Results: Ninety-three percent of school-age children were found Vitamin D deficient as per serum 25(OH) D levels of <20 ng/ml. The prevalence was significantly higher among females. Conclusion: A high prevalence of VDD was found in children residing in high altitude region. PMID:28217519

  13. Small RNA profiling reveals phosphorus deficiency as a contributing factor in symptom expression for citrus huanglongbing disease.

    Science.gov (United States)

    Zhao, Hongwei; Sun, Ruobai; Albrecht, Ute; Padmanabhan, Chellappan; Wang, Airong; Coffey, Michael D; Girke, Thomas; Wang, Zonghua; Close, Timothy J; Roose, Mikeal; Yokomi, Raymond K; Folimonova, Svetlana; Vidalakis, Georgios; Rouse, Robert; Bowman, Kim D; Jin, Hailing

    2013-03-01

    Huanglongbing (HLB) is a devastating citrus disease that is associated with bacteria of the genus 'Candidatus Liberibacter' (Ca. L.). Powerful diagnostic tools and management strategies are desired to control HLB. Host small RNAs (sRNA) play a vital role in regulating host responses to pathogen infection and are used as early diagnostic markers for many human diseases, including cancers. To determine whether citrus sRNAs regulate host responses to HLB, sRNAs were profiled from Citrus sinensis 10 and 14 weeks post grafting with Ca. L. asiaticus (Las)-positive or healthy tissue. Ten new microRNAs (miRNAs), 76 conserved miRNAs, and many small interfering RNAs (siRNAs) were discovered. Several miRNAs and siRNAs were highly induced by Las infection, and can be potentially developed into early diagnosis markers of HLB. miR399, which is induced by phosphorus starvation in other plant species, was induced specifically by infection of Las but not Spiroplasma citri that causes citrus stubborn-a disease with symptoms similar to HLB. We found a 35% reduction of phosphorus in Las-positive citrus trees compared to healthy trees. Applying phosphorus oxyanion solutions to HLB-positive sweet orange trees reduced HLB symptom severity and significantly improved fruit production during a 3-year field trial in south-west Florida. Our molecular, physiological, and field data suggest that phosphorus deficiency is linked to HLB disease symptomology.

  14. Does FXIII deficiency impair wound healing after myocardial infarction?

    Directory of Open Access Journals (Sweden)

    Matthias Nahrendorf

    Full Text Available Inadequate healing of myocardial infarction may contribute to local expansion of the infarct, frequently leading to chamber dilation, heart failure, or myocardial rupture. Experimental evidence in mouse models suggests that Factor XIII might play a key role in wound healing, and low persistent values lead to increased incidence of cardiac rupture following myocardial infarction. Here we would like to share our initial clinical experiences with strikingly similar observations in patients with this grave disease, and compare these observations to experimental findings.

  15. Ressenya a Ovidio Cuella Esteban i Juan B. Simó Castillo, Bulario de Benedicto XIII (1394-1423. VI. Diócesis de Tortosa. Maestrazgo de Santa María de Montesa. Testamento de Benedicto XIII, Peníscola, Associació ‘Amics del Papa Luna’, 2013, pp. 680, ISBN 978-84-616-7651-4

    Directory of Open Access Journals (Sweden)

    Blai Josep Server Server

    2014-12-01

    Full Text Available Review to Ovidio Cuella Esteban i Juan B. Simó Castillo, Bulario de Benedicto XIII (1394-1423. VI. Diócesis de Tortosa. Maestrazgo de Santa María de Montesa. Testamento de Benedicto XIII, Peníscola, Associació ‘Amics del Papa Luna’, 2013, pp. 680, ISBN 978-84-616-7651-4

  16. Alpha1-antitrypsin deficiency

    DEFF Research Database (Denmark)

    Stolk, Jan; Seersholm, Niels; Kalsheker, Noor

    2006-01-01

    biennially to exchange views and research findings. The fourth biennial meeting was held in Copenhagen, Denmark, on 2-3 June 2005. This review covers the wide range of AAT deficiency-related topics that were addressed encompassing advances in genetic characterization, risk factor identification, clinical...... epidemiology, inflammatory and signalling processes, therapeutic advances, and lung imaging techniques....

  17. Acquired factor XII deficiency in a woman with recurrent pregnancy loss: working on a differential diagnosis in a single case

    Directory of Open Access Journals (Sweden)

    Di Micco Pierpaolo

    2005-12-01

    Full Text Available Abstract Background Antiphospholipid syndrome (APS has been often associated to RPL since 1980 and some reports in the Literature rarely described antibodies to factor XII in patients with APS. Case history We report the case history of 34-year-old caucasian women with recurrent fetal loss and persistent prolonged activated partial thromboplastin time. Haemostatic tests revealed persistent light decrease of clotting factor XII with normal values of IgG and IgM anticardiolipin antibodies and transient positivity for lupus anticoagulant (LA. Few reports in the Literature described antibodies to factor XII in patient with antiphospholipid syndrome (APS and transient LA. So, once other causes of RPL were excluded, the patient was diagnosed an unusual form of APS associated to antibodies to factor XII, reduced factor XII plasma levels, transient LA and prolonged activated partial thromboplastin time. Discussion We suggest to consider also antibodies directed to clotting factors (e.g. factor XII in our case as second step of thrombophilia screening in RPL, in particular if a persistent prolonged aPTT is present without an apparent cause.

  18. Identidad de las hospitalarias en la corona de Aragón (siglos XII-XIII

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    Maria Bonet Donato

    2014-12-01

    Full Text Available La rama femenina del Hospital fue singular en el seno de la institución y logró una autonomía y una entidad diferenciadora que se manifestó y consolidó en la afirmación de elementos identitarios. La identidad de las hospitalarias se concretó en la reivindicación y el despliegue de funciones y actividades singulares, en la existencia de mujeres carismáticas que encarnaron dicha identidad y en el reconocimiento de ciertos espacios. Las comunidades de monjas hospitalarias de la corona de Aragón en los siglos XII y XIII se caracterizaron por una diferenciación entre ellas mayor que la que se dio entre las encomiendas masculinas, y a su vez tuvieron mayor individualización en el contexto institucional. Las hospitalarias expresaron su espiritualidad y lograron visibilidad y protagonismo social, conforme a la creación o recreación de la identidad hospitalaria. Dicha identidad femenina hospitalaria resultó de la combinación de aspectos concernientes a la identidad familiar, femenina, espiritual, religiosa, individual, política, y se acomodó a la “personalidad” de cada entidad conventual, e inclusive a la de algunas figuras con destacado liderazgo

  19. Tres Violantes: las mujeres de una familia en el poder a lo largo del siglo XIII

    Directory of Open Access Journals (Sweden)

    Fuente Pérez, María Jesús

    2016-06-01

    Full Text Available The power of women, in particular the power of queens, has been a topic of great interest to historians, medievalists in particular; though much has been written about the subject, many questions remain. “Three Violantes” will look at the role of two Iberian queens and one Spanish princess in the thirteenth century Iberian kingdoms. In England and in other European kingdoms of that time, queens had lost authority and their agency was limited to the power of intercession and mediation, I will try to determine whether this also holds true for the Iberian queens.El interés por el tema del poder de las mujeres, en particular de las reinas, ha planteado muchos interrogantes sobre la naturaleza de ese poder. “Tres Violantes” examina la participación de dos reinas y una infanta de una misma familia, en aspectos diversos de la vida de las coronas de Aragón y Castilla en el siglo XIII, con el objetivo de apreciar si el papel de las reinas hispanas, en particular su participación política, siguió un camino similar al de las reinas de ese tiempo que, como bien se ha estudiado para las inglesas, fueron perdiendo autoridad y se vieron limitadas a ejercer poder a través de la intercesión y mediación.

  20. Emission Lines of Fe XI - XIII in the Extreme Ultraviolet Region

    Science.gov (United States)

    Lepson, Jaan; Beiersdorfer, Peter; Liedahl, Duane; Desai, Priya; Brickhouse, Nancy; Dupree, Andrea; Kahn, Steven

    2009-05-01

    Iron is one of the most abundant heavy elements in extreme ultraviolet spectra of astrophysical and laboratory plasmas, and its various ions radiate profusely in the extreme ultraviolet (EUV) wavelength band. Iron emission in the EUV provides important d iagnostic tools for such properties as plasma temperature and density, and perhaps even magnetic field strength. Despite its importance to astrophysics and magnetic fusion, knowledge of the EUV spectrum of iron is incomplete. Identification of iron emis sion lines is hampered by the paucity of accurate laboratory measurements and the uncertainty of even the best atomic models. As part of a project to measure and compile emission line data in the EUV, we present here spectra and lines of Fe XI - XIII recorded on the Livermore EBIT-II electron beam ion trap in the 50 - 120 åregion. We measured line positions to 0.02 åand relative intensities with an accuracy of one part in twenty. Many new lines are identified and added to the available databa ses. Part of this work was performed under the auspices of the U S Department of Energy by Lawrence Livermore National Laboratory under Contract DE-AC52-07NA27344 and was supported by NASA's Astronomy and Physics Research and Analysis Program under Con t ract NNH07AF811.

  1. Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Patschan Daniel

    2011-12-01

    Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

  2. Effects of octreotide on insulin-like growth factor I and metabolic indices in growth hormone-treated growth hormone-deficient patients

    DEFF Research Database (Denmark)

    Laursen, Torben; Jørgensen, Jens Otto Lunde; Ørskov, Hans;

    1993-01-01

    Abstract Animals studies have demonstrated that in addition to inhibiting growth hormone (GH) secretion octreotide inhibits in a direct manner hepatic or peripheral insulin-like growth factor I (IGF-I) generation. To test this hypothesis in humans we studied ten GH-deficient patients with frequent.......5 +/- 1.47 (octreotide) and 12.8 +/- 1.42 (placebo) (p = 0.83), and Tmax (h) was 6.1 +/- 0.97 (octreotide) and 5.2 +/- 0.65 (placebo) (p = 0.49). Growth hormone administration was associated with an increase in serum IGF-I (microgram/l), which was identical during the two studies, from 85.3 +/- 19...

  3. Free insulin-like growth factor I serum levels in 1430 healthy children and adults, and its diagnostic value in patients suspected of growth hormone deficiency

    DEFF Research Database (Denmark)

    Juul, A; Holm, K; Kastrup, K W;

    1997-01-01

    Serum levels of total insulin-like growth factor I (IGF-I) and IGF-binding protein-3 (IGFBP-3) reflect endogenous GH secretion in healthy children, which makes them good diagnostic markers for screening of GH deficiency (GHD) in short children, although some controversy still exists. Only a minor...... determinations in patients suspected of GHD has only been reported in a few studies, whereas no previous reports on the diagnostic value of free IGF-I levels in adults suspected of GHD exist. Serum levels of free IGF-I were determined in 1430 healthy children, adolescents, and adults by a newly developed......, commercially available immunoradiometric assay (Diagnostic Systems Laboratories) to establish valid normative data for this analysis. We studied the diagnostic value of free IGF-I in relation to total IGF-I and IGFBP-3 determinations in adults who were suspected of GHD. A GH provocative test, using oral...

  4. Impact of tumor necrosis factor receptor p55 deficiency in susceptibility of C57BL/6 mice to infection with Leishmania (Leishmania) amazonensis.

    Science.gov (United States)

    Cargnelutti, Diego Esteban; Salomón, María Cristina; Celedon, Verónica; Cuello-Carrión, Fernando Darío; Gea, Susana; Di Genaro, María Silvia; Scodeller, Eduardo Alberto

    2016-04-01

    Tumor necrosis factor (TNF) is involved in host resistance to several intracellular pathogens. Although the critical role of TNF receptor (TNFR)p55 in Leishmania (Leishmania) major infection has been demonstrated, the impact of TNFRp55 deficiency on L. (L.) amazonensis infection has not been explored. L. (L.) amazonensis-infected TNFRp55(-/-) mice failed to resolve lesions, whereas C57BL/6 wild-type mice completely healed. The susceptibility of the TNFRp55(-/-) mice was characterized by higher lesion size and histopathological damage in comparison with the wild-type mice. A marked increased of the splenic index was observed in the TNFRp55(-/-) mice after 15 weeks infection. These results show that in the absence of TNFRp55, L. (L.) amazonensis-infected knockout mice fail to resolve lesions, whereas wild-type mice completely heal.

  5. Small RNA Profiling Reveals Phosphorus Deficiency as a Contributing Factor in Symptom Expression for Citrus Huanglongbing Disease

    Institute of Scientific and Technical Information of China (English)

    Hongwei Zhao; Ruobai Sun; Ute Albrecht; Chellappan Padmanabhan; Airong Wang; Michael D.Coffey; Thomas Girke

    2013-01-01

    Huanglongbing (HLB) is a devastating citrus disease that is associated with bacteria of the genus "Candidatus Liberibacter' (Ca.L.).Powerful diagnostic tools and management strategies are desired to control HLB.Host small RNAs (sRNA) play a vital role in regulating host responses to pathogen infection and are used as early diagnostic markers for many human diseases,including cancers.To determine whether citrus sRNAs regulate host responses to HLB,sRNAs were profiled from Citrus sinensis 10 and 14 weeks post grafting with Ca.L.asiaticus (Las)-positive or healthy tissue.Ten new microRNAs (miRNAs),76 conserved miRNAs,and many small interfering RNAs (siRNAs) were discovered.Several miRNAs and siRNAs were highly induced by Las infection,and can be potentially developed into early diagnosis markers of HLB.miR399,which is induced by phosphorus starvation in other plant species,was induced specifically by infection of Las but not Spiroplasma citri that causes citrus stubborn--a disease with symptoms similar to HLB.We found a 35% reduction of phosphorus in Las-positive citrus trees compared to healthy trees.Applying phosphorus oxyanion solutions to HLB-positive sweet orange trees reduced HLB symptom severity and significantly improved fruit production during a 3-year field trial in south-west Florida.Our molecular,physiological,and field data suggest that phosphorus deficiency is linked to HLB disease symptomology.

  6. Growth hormone (GH) treatment increases serum insulin-like growth factor binding protein-3, bone isoenzyme alkaline phosphatase and forearm bone mineral content in young adults with GH deficiency of childhood onset

    DEFF Research Database (Denmark)

    Juul, A; Pedersen, S A; Sørensen, S

    1994-01-01

    Recent studies have demonstrated that growth hormone (GH)-deficient adults have a markedly decreased bone mineral content compared to healthy adults. However, there are conflicting results regarding the effects of GH treatment on bone mineral content in GH-deficient adults. Therefore, we evaluated...... the effect of GH treatment on a marker of bone formation (bone alkaline phosphatase), hepatic excretory function and distal forearm bone mineral content in GH-deficient adults. Growth hormone was administered subcutaneously in 21 adults (13 males and 8 females) with GH deficiency of childhood onset for 4...... months in a double-blind, placebo-controlled GH trial, while 13 of the patients then received further GH for an additional 14 months. Serum insulin-like growth factor I (IGF-I) increased significantly from 100 to 279 micrograms/l and IGF binding protein-3 (IGFBP-3) from 1930 to 3355 micrograms/l after 4...

  7. Protein replacement therapy and gene transfer in canine models of hemophilia A, hemophilia B, von willebrand disease, and factor VII deficiency.

    Science.gov (United States)

    Nichols, Timothy C; Dillow, Aaron M; Franck, Helen W G; Merricks, Elizabeth P; Raymer, Robin A; Bellinger, Dwight A; Arruda, Valder R; High, Katherine A

    2009-01-01

    Dogs with hemophilia A, hemophilia B, von Willebrand disease (VWD), and factor VII deficiency faithfully recapitulate the severe bleeding phenotype that occurs in humans with these disorders. The first rational approach to diagnosing these bleeding disorders became possible with the development of reliable assays in the 1940s through research that used these dogs. For the next 60 years, treatment consisted of replacement of the associated missing or dysfunctional protein, first with plasma-derived products and subsequently with recombinant products. Research has consistently shown that replacement products that are safe and efficacious in these dogs prove to be safe and efficacious in humans. But these highly effective products require repeated administration and are limited in supply and expensive; in addition, plasma-derived products have transmitted bloodborne pathogens. Recombinant proteins have all but eliminated inadvertent transmission of bloodborne pathogens, but the other limitations persist. Thus, gene therapy is an attractive alternative strategy in these monogenic disorders and has been actively pursued since the early 1990s. To date, several modalities of gene transfer in canine hemophilia have proven to be safe, produced easily detectable levels of transgene products in plasma that have persisted for years in association with reduced bleeding, and correctly predicted the vector dose required in a human hemophilia B liver-based trial. Very recently, however, researchers have identified an immune response to adeno-associated viral gene transfer vector capsid proteins in a human liver-based trial that was not present in preclinical testing in rodents, dogs, or nonhuman primates. This article provides a review of the strengths and limitations of canine hemophilia, VWD, and factor VII deficiency models and of their historical and current role in the development of improved therapy for humans with these inherited bleeding disorders.

  8. VLCAD deficiency

    DEFF Research Database (Denmark)

    Boneh, A; Andresen, B S; Gregersen, N

    2006-01-01

    We diagnosed six newborn babies with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) through newborn screening in three years in Victoria (prevalence rate: 1:31,500). We identified seven known and two new mutations in our patients (2/6 homozygotes; 4/6 compound heterozygotes). Blood...

  9. Dwarfism and impaired gut development in insulin-like growth factor II mRNA-binding protein 1-deficient mice

    DEFF Research Database (Denmark)

    Hansen, Thomas V O; Hammer, Niels A; Nielsen, Jacob

    2004-01-01

    Insulin-like growth factor II mRNA-binding protein 1 (IMP1) belongs to a family of RNA-binding proteins implicated in mRNA localization, turnover, and translational control. Mouse IMP1 is expressed during early development, and an increase in expression occurs around embryonic day 12.5 (E12.5). T...

  10. A Common Susceptibility Factor of Both Autism and Epilepsy: Functional Deficiency of GABA[subscript A] Receptors

    Science.gov (United States)

    Kang, Jing-Qiong; Barnes, Gregory

    2013-01-01

    Autism and epilepsy are common childhood neurological disorders with a great heterogeneity of clinical phenotypes as well as risk factors. There is a high co-morbidity of autism and epilepsy. The neuropathology of autism and epilepsy has similar histology implicating the processes of neurogenesis, neural migration, programmed cell death, and…

  11. PREVALENCE AND FACTORS ASSOCIATED WITH VITAMIN B12 DEFICIENCY IN ELDERLY FROM VIÇOSA/MG, BRASIL.

    Science.gov (United States)

    Oliveira Martinho, Karina; Luiz Araújo Tinôco, Adelson; Queiroz Ribeiro, Andréia

    2015-11-01

    La prevalencia de deficiencia nutricional de vitamina B12 aumenta con la edad y es especialmente común en la población mayor. El objetivo de este estudio consistió en determinar su prevalencia y los factores asociados a esta carencia en mayores no institucionalizados de Viçosa, Estado de Minas Gerais, Brasil. Métodos: estudio poblacional interseccional, realizado para identificar la prevalencia y los factores asociados a deficiencia de vitamina B12 entre la población de mayores en Viçosa (MG). Los datos fueron recopilados desde agosto de 2011 hasta junio de 2012 mediante una encuesta en los hogares y pruebas hematológicas y bioquímicas realizadas en 340 mayores. Resultados: la prevalencia de deficiencia de vitamina B12 en este grupo fue del 17,4% (95% CI, 13,4% - 21,4%). La deficiencia cognitiva se presenta como un factor importante relacionado con la deficiencia de vitamina B12. Conclusiones: el informe actual contribuye a los estudios que destacan ciertos factores que podrían afectar al rendimiento de las personas mayores en su proceso de envejecimiento natural, especialmente cuando estos factores están asociados con deficiencia cognitiva y dan lugar a una discapacidad significativa así como pérdida de calidad de vida. Así, los resultados aquí presentados han servido para aportar un conocimiento más comprensivo sobre la relación entre deficiencia de B12 y su impacto sobre este grupo de población. También han demostrado su relevancia de cara a la planificación de programas e iniciativas de salud pública centrados en este grupo de población.

  12. Prevalence of prenatal zinc deficiency and its association with socio-demographic, dietary and health care related factors in Rural Sidama, Southern Ethiopia: A cross-sectional study

    Directory of Open Access Journals (Sweden)

    Gebremedhin Samson

    2011-11-01

    Full Text Available Abstract Background Several studies witnessed that prenatal zinc deficiency (ZD predisposes to diverse pregnancy complications. However, scientific evidences on the determinants of prenatal ZD are scanty and inconclusive. The purpose of the present study was to assess the prevalence and determinants of prenatal ZD in Sidama zone, Southern Ethiopia. Methods A community based, cross-sectional study was conducted in Sidama zone in January and February 2011. Randomly selected 700 pregnant women were included in the study. Data on potential determinants of ZD were gathered using a structured questionnaire. Serum zinc concentration was measured using Atomic Absorption Spectrometry. Statistical analysis was done using logistic regression and linear regression. Results The mean serum zinc concentration was 52.4 (+/-9.9 μg/dl (95% CI: 51.6-53.1 μg/dl. About 53.0% (95% CI: 49.3-56.7% of the subjects were zinc deficient. The majority of the explained variability of serum zinc was due to dietary factors like household food insecurity level, dietary diversity and consumption of animal source foods. The risk of ZD was 1.65 (95% CI: 1.02-2.67 times higher among women from maize staple diet category compared to Enset staple diet category. Compared to pregnant women aged 15-24 years, those aged 25-34 and 35-49 years had 1.57 (95% CI: 1.04-2.34 and 2.18 (95% CI: 1.25-3.63 times higher risk of ZD, respectively. Women devoid of self income had 1.74 (95% CI: 1.11-2.74 time increased risk than their counterparts. Maternal education was positively associated to zinc status. Grand multiparas were 1.74 (95% CI: 1.09-3.23 times more likely to be zinc deficient than nulliparas. Frequency of coffee intake was negatively association to serum zinc level. Positive association was noted between serum zinc and hemoglobin concentrations. Altitude, history of iron supplementation, maternal workload, physical access to health service, antenatal care and nutrition education were

  13. Activation of the hypoxia-inducible factor pathway induced by prolyl hydroxylase domain 2 deficiency enhances the effect of running training in mice.

    Science.gov (United States)

    Nunomiya, A; Shin, J; Kitajima, Y; Dan, T; Miyata, T; Nagatomi, R

    2017-05-01

    Hypoxic response mediated by hypoxia-inducible factor (HIF) seems to contribute to the benefit of endurance training. To verify the direct contribution of HIF activation to running training without exposure to atmospheric hypoxia, we used prolyl hydroxylase domain 2 (PHD2) conditional knockout mice (cKO), which exhibit HIF activation independent of oxygen concentration, and we examined their maximal exercise capacity before and after 4 weeks of treadmill exercise training. Phd2(f/f) mice (n = 26) and Phd2 cKO mice (n = 24) were randomly divided into two groups, trained and untrained, and were subjected to maximal running test before and after a 4-week treadmill-training regimen. Prolyl hydroxylase domain 2 deficiency resulted in HIF-α protein accumulation. Phd2 cKO mice exhibited marked increases in haematocrit values and haemoglobin concentrations, as well as an increase in the capillary number in the skeletal muscle. The 4-week training elicited an increase in the capillary-to-fibre (C/F) ratio and succinyl dehydrogenase activity of the skeletal muscle. Importantly, trained Phd2 cKO mice showed a significantly greater improvement in running time than trained control mice (P training and the activation of the HIF pathway are important for maximizing the effect of running training. We conclude that the activation of the HIF pathway induced by PHD2 deficiency enhances the effect of running training. © 2016 The Authors. Acta Physiologica published by John Wiley & Sons Ltd on behalf of Scandinavian Physiological Society.

  14. Lipoxin A4 and platelet activating factor are involved in E. coli or LPS-induced lung inflammation in CFTR-deficient mice.

    Directory of Open Access Journals (Sweden)

    Haiya Wu

    Full Text Available CFTR (cystic fibrosis transmembrane conductance regulator is expressed by both neutrophils and platelets. Lack of functional CFTR could lead to severe lung infection and inflammation. Here, we found that mutation of CFTR (F508del or inhibition of CFTR in mice led to more severe thrombocytopenia, alveolar neutrocytosis and bacteriosis, and lower lipoxin A4/MIP-2 (macrophage inhibitory protein-2 or lipoxin A4/neutrophil ratios in the BAL (bronchoalveolar lavage during acute E. coli pneumonia. In vitro, inhibition of CFTR promotes MIP-2 production in LPS-stimulated neutrophils; however, lipoxin A4 could dose-dependently suppress this effect. In LPS-induced acute lung inflammation, blockade of PSGL-1 (P-selectin glycoprotein ligand-1 or P-selectin, antagonism of PAF by WEB2086, or correction of mutated CFTR trafficking by KM11060 could significantly increase plasma lipoxin A4 levels in F508del relevant to wildtype mice. Concurrently, F508del mice had higher plasma platelet activating factor (PAF levels and PAF-AH activity compared to wildtype under LPS challenge. Inhibiting hydrolysis of PAF by a specific PAF-AH (PAF-acetylhydrolase inhibitor, MAFP, could worsen LPS-induced lung inflammation in F508del mice compared to vehicle treated F508del group. Particularly, depletion of platelets in F508del mice could significantly decrease plasma lipoxin A4 and PAF-AH activity and deteriorate LPS-induced lung inflammation compared to control F508del mice. Taken together, lipoxin A4 and PAF are involved in E. coli or LPS-induced lung inflammation in CFTR-deficient mice, suggesting that lipoxin A4 and PAF might be therapeutic targets for ameliorating CFTR-deficiency deteriorated lung inflammation.

  15. Selective cognitive deficits and reduced hippocampal brain-derived neurotrophic factor mRNA expression in small-conductance calcium-activated K+ channel deficient mice.

    Science.gov (United States)

    Jacobsen, J P R; Redrobe, J P; Hansen, H H; Petersen, S; Bond, C T; Adelman, J P; Mikkelsen, J D; Mirza, N R

    2009-09-29

    Small-conductance calcium-activated K(+) channels 1-3 (SK1-3) are important for neuronal firing regulation and are considered putative CNS drug targets. For instance non-selective SK blockers improve performance in animal models of cognition. The SK subtype(s) involved herein awaits identification and the question is difficult to address pharmacologically due to the lack of subtype-selective SK-channel modulators. In this study, we used doxycycline-induced conditional SK3-deficient (T/T) mice to address the cognitive consequences of selective SK3 deficiency. In T/T mice SK3 protein is near-eliminated from the brain following doxycycline treatment. We tested T/T and wild type (WT) littermate mice in five distinct learning and memory paradigms. In Y-maze spontaneous alternations and five-trial inhibitory avoidance the performance of T/T mice was markedly inferior to WT mice. In contrast, T/T and WT mice performed equally well in passive avoidance, object recognition and the Morris water maze. Thus, some aspects of working/short-term memory are disrupted in T/T mice. Using in situ hybridization, we further found the cognitive deficits in T/T mice to be paralleled by reduced brain-derived neurotrophic factor (BDNF) mRNA expression in the dentate gyrus and CA3 of the hippocampus. BDNF mRNA levels in the frontal cortex were not affected. BDNF has been crucially implicated in many cognitive processes. Hence, the biological substrate for the cognitive impairments in T/T mice could conceivably entail reduced trophic support of the hippocampus.

  16. Valor y sentido del conocimiento en las órdenes mendicantes del siglo XIII

    Directory of Open Access Journals (Sweden)

    Gerald Cresta

    2010-07-01

    Full Text Available El siglo XIII se expresa por medio de un concepto propio de la Universidad en que todo el saber debía estar orientado a la sapientia sacrae paginae, que fue expresada por la teología. Esta cuestión representaba sin duda un ideal de la Iglesia, pero asimismo un desafíoespeculativo para los pensadores de entonces. Con la complejidad epistemológica heredada del siglo XII y la inserción de las órdenes mendicantes en la labor académica, principalmente dominicos y franciscanos, esta fase de la historia de las ideas asistía a unantagonismo fundamental: el naturalismo de la herencia clásica - junto con el aporte del pensamiento árabe - y el humanismo cristiano se disputan la explicación del fin último del hombre. El valor y el sentido del conocimiento dependería en gran medida de la respuestadada a esta diversidad de orientaciones. Y si bien es un hecho clave la influencia de los grandes autores antiguos, aristotelismo y neoplatonismo no son prerrogativa de una y otra orden. En los escritos de Santo Tomás está presente el neoplatonismo y es asimismosignificativa la orientación empírica de la escuela franciscana en Inglaterra. Unos y otros, sin embargo, concordarían en una unidad del saber que podríamos interpretar bajo el signo de los trascendentales del ser.O século XIII manifesta-se por meio de uma formulação própria da Universidade, em que todo o conhecimento deve ser orientado na sapientia sacrae paginae, que foi expressa pela teologia. Esta questão foi, sem dúvida, um ideal da Igreja, mas também um desafioespeculativo para pensadores da época. Com a complexidade epistemológica herdada do século XII e a inclusão das ordens mendicantes no trabalho acadêmico, principalmente Dominicanos e Franciscans, esta fase da história da idéias assiste ao antagonismo básico: o naturalismo da herança clássica - com a contribuição do pensamento árabe - e o Humanismo Cristão que concorrerem para a explicação da meta final do

  17. PREFACE: MCWASP XIII: International Conference on Modeling of Casting, Welding and Advanced Solidification Processes

    Science.gov (United States)

    Ludwig, Andreas

    2012-07-01

    Due to fast-paced development in computer technologies during the last three decades, computer-based process modeling has become an important tool for the improvement of existing process technologies and the development of new, innovative technologies. With the help of numerical process simulations, complex and costly experimental trials can now be reduced to a minimum. For metallurgical processes in particular, computer simulations are of outstanding importance, as the flow and solidification of molten alloys or the formation of microstructure and defects can hardly be observed experimentally. Corresponding computer simulations allow us inside views into the key process phenomena and so offer great potential for optimization. In 1980 the conference series 'Modeling of Casting, Welding and Advanced Solidification Processes (MCWASP)' was started up, and has now been continued by holding the 13th international conference on 'Modeling of Casting, Welding and Advanced Solidification Processes', MCWASP XIII, in Schladming, Austria, from June 17-22 2012. Around 200 scientists from industry and academia, coming from 20 countries around the globe attended 78 oral and 50 poster presentations on different aspects of solidification-related modeling topics. Besides process-related sessions such as (i) Ingot and Shape Casting, (ii) Continuous Casting and Direct Chill Casting, (iii) Directional Solidification and Zone Melting, (iv) Welding, and (v) Centrifugal Casting, a larger focus was put on (vi) Experimental Investigation and In-Situ Observations. In recent years, this topic has been significantly strengthened as advanced synchrotron technologies allow fantastic in-situ observations of phenomena happening inside small metallic samples. These observations will definitely serve as a benchmark for the modeling community. Further macroscopic aspects of advanced solidification science were tackled in the sessions (vii) Electromagnetic Coupling, (viii) Thermomechanics, (ix

  18. Fronteras de aire. Portugal, León y Castilla en el siglo XIII

    Directory of Open Access Journals (Sweden)

    Romero Portilla, Paz

    2015-12-01

    Full Text Available Studied documents illustrate a close link between the kingdom of Portugal and the kingdoms of Castile and Leon in the 13th century. They demonstrate a diplomatic tradition that tended to guarantee a balance of power between them in spite of events that would eventually divide them, such as the union of Castile and Leon. This century was a difficult one for the three kingdoms due to wars, child monarchs, problems with the nobility, territorial and border consolidation, etc. Nevertheless, aspects related to history, geography, society, family, the economy and the church, well reflected in these documents were without doubt, a driving force behind the unique relationship between the kingdoms. A preliminary glance at these documents illustrates the issues that both united and separated Portugal with Leon and Castile at the height of the medieval period.La documentación estudiada refl eja una estrecha vinculación del reino de Portugal con León y Castilla en el siglo XIII. Nos muestra una trayectoria diplomática que tiende a garantizar el equilibrio entre los reinos peninsulares, a pesar de acontecimientos que lo rompen, como la unión de Castilla y León. Difícil siglo para los tres reinos debido a guerras, minorías de edad, problemas con la nobleza, afianzamiento territorial y fronterizo, etc. Pero aspectos históricos, geográficos, sociales, familiares, económicos y eclesiásticos, bien reflejados en los documentos, motivaron, sin duda, una especial relación entre los reinos. Un primer acercamiento a la documentación nos ha evidenciado cuáles fueron los asuntos que vincularon y separaron a Portugal de León y Castilla en la plenitud medieval. [gl] A documentación estudada reflicte unha estreita vinculación do reino de Portugal con León e Castela no século XIII. Móstranos unha traxectoria diplomática que tende a garantir o equilibrio entre os reinos peninsulares, a pesar de acontecementos que o rompen, como a unión de Castela e Le

  19. [Religion and healing in the XIII century: some considerations about therapy and holy medical observations in Iacopo da Varagine's Cronica Civitatis Ianuensis].

    Science.gov (United States)

    Moggia, Carlo

    2004-01-01

    The article is dedicated to recovery and holy therapy in Medieval society. In the XIII century Roman Church define the religious observations of physical and spiritual healing: the intervention of Saints and their holy miracles is very important for a perfect recovery. The article analyse the holy therapy through the study of the Cronica Civitatis Ianuensis read by the Dominican friar Iacopo da Varagine, at the end of the XIII century. This work represents one of the most important literary and hagiographic instrument to comprehend medical medieval imaginary.

  20. What to look for beyond "pathogenic" factors in senile dementia? A functional deficiency of Ca²⁺ signaling.

    Science.gov (United States)

    Chen, Ming; Nguyen, Huey T; Sawmiller, Darrell R

    2011-01-01

    We have contended that senile conditions--illnesses after age 60 and fully age-penetrating, such as tooth, hearing or memory loss--are not distinct "diseases" in medical nature, because they are caused by aging. Since the pace of aging varies among individuals and is much influenced by risk factors, senile conditions will only affect some but not all elderly. However, perhaps due to its unusually heavy burdens and tremendous social pressures, senile dementia (SD) has been singled out from other senile conditions and redefined as a curable "disease" (Alzheimer's). This highly popular definition has thus opened a Pandora's box that has been confusing us up until now and warrants further scrutiny. In this article we discuss: a) what should we logically look for in SD beyond "pathogenic" factors?; b) why Ca2+, a central regulator in neurotransmission, should be the primary player in SD; c) why the functionality of Ca2+ signaling, or its vibrant wave frequency and amplitude, must undergo down-regulation during aging, though this is intriguingly accompanied by an increase of Ca2+ "levels"; d) why intervention for SD should target Ca2+ function by promoting energy metabolisms and by Ca2+ agonists such as caffeine and nicotine, but not by "antagonists" as widely believed; and e) why our study should focus on aging, not "cell death", a seemingly attractive paradigm but perhaps too late for intervention. We also seek answers for why unproven hypotheses can become dogmas and inhibit self-correcting mechanisms of science.

  1. Failure of adrenal corticosterone production in POMC-deficient mice results from lack of integrated effects of POMC peptides on multiple factors.

    Science.gov (United States)

    Karpac, Jason; Czyzewska, Katarzyna; Kern, Andras; Brush, Richard S; Anderson, Robert E; Hochgeschwender, Ute

    2008-08-01

    Production of corticosteroids from the adrenal gland is a multistep process in which corticosterone is enzymatically processed from its precursor cholesterol. The main hormone regulating the production of corticosterone is the proopiomelanocortin (POMC)-derived adrenocorticotropic hormone (ACTH). Adrenals of POMC-deficient (POMC(-/-)) mice do not produce corticosterone either at basal levels or in response to acute stimulation with ACTH. However, pharmacological amounts of ACTH delivered continuously elicit corticosterone production over time. To define the relative effects of ACTH on individual factors involved in corticosterone production, parameters of adrenal cholesterol metabolism and steroidogenesis were examined in POMC(-/-) mice compared with wild-type and ACTH-treated mutant mice. POMC(-/-) adrenals lack cholesterol esters (CE); adrenal CE is restored with ACTH treatment. However, discontinuation of ACTH treatment stops corticosterone production despite the presence of adrenal CE. Failure of corticosterone production by POMC(-/-) adrenals occurs despite the constitutive presence of transcripts of genes required for cholesterol metabolism and steroidogenesis. Levels of key proteins involved in selective cholesterol uptake and steroidogenesis were attenuated; ACTH treatment increased these protein levels, most significantly those of the receptor responsible for selective uptake of CE, scavenger receptor class B, type I (SR-BI). Our studies reveal that failure of corticosterone production of POMC(-/-) adrenal glands and its pharmacological reconstitution by ACTH are not mediated by any one individual protein, but rather as an integrated effect on multiple factors from import of the substrate cholesterol to its conversion to corticosterone.

  2. [Zinc deficiency and associated factors in colombian children; results from the 2010 national nutrition survey; a cross sectional study].

    Science.gov (United States)

    Martínez-Torres, Javier; Ramírez-Vélez, Robinson

    2014-04-01

    Introducción: El zinc (Zn) es un micronutriente esencial en el crecimiento celular, la síntesis proteica y la diferenciación celular. La deficiencia de Zn afecta el crecimiento y desarrollo del niño, el metabolismo energético y la respuesta inmune. Objetivo: Examinar los factores asociados a la deficiencia de Zn en una muestra representativa de colombianos niños. Pacientes y métodos: Estudio descriptivo transversal, secundario de la información obtenida en la Encuesta Nacional de la Situación Nutricional 2010 (ENSIN 2010), en 4.279 niños entre 12 y 59 meses. Los niveles plasmáticos de Zn se determinaron por espectrofotometría de absorción atómica, y los factores asociados (sexo, edad, etnia, puntaje de SISBEN, región y área geográfica) se recogieron por encuesta estructurada. Se establecieron asociaciones mediante la construcción de modelos de regresión y factores asociados. Resultados: Se encontró un valor promedio de Zinc de 78,5 μg/dl (IC 95% = 76,7-80,4 μg/dl). El 43,3% (IC 95% 42,2-44,3%) de los niños presentaron niveles de Zn menores a 65 μg/dl. Los niños pertenecientes a grupos étnicos (indígena) o que residen en áreas rurales; presentaron mayor déficit de Zn (56,3% y 47,8%) respectivamente. Los modelos de regresión muestran que; ser indígena (OR 1,76 IC 95% 1,29-2,41); y residir en zonas rurales (OR 1,39 IC 95% 1,16-1,67), se asociaron al déficit de Zn. Conclusiones: La población estudiada presenta una alta prevalencia de déficit de Zn, por lo que se recomienda intervenciones integrales donde estén involucrados el componente nutricional y educativo.

  3. Growth hormone (GH) provocative retesting of 108 young adults with childhood-onset GH deficiency and the diagnostic value of insulin-like growth factor I (IGF-I) and IGF-binding protein-3

    DEFF Research Database (Denmark)

    Juul, A; Kastrup, K W; Pedersen, S A;

    1997-01-01

    Serum levels of total insulin-like growth factor I (IGF-I) and IGF-binding protein-3 (IGFBP-3) reflect the endogenous GH secretion in healthy children and exhibit little diurnal variation, which makes them good diagnostic markers for screening of GH deficiency (GHD) in short children, although so...

  4. Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study

    NARCIS (Netherlands)

    H.L.A. Janssen (Harry); J.P. Vandenbroucke; F.R. Rosendaal (Frits); B. van Hoek (Bart); J.R. Meinardi; F.P. Vleggaar (Frank); S.H. van Uum; E.B. Haagsma (Els); F.J.M. van der Meer; J. van Hattum (Jan); R.A. Chamuleau; R.P.R. Adang (Rob)

    2000-01-01

    textabstractIn a collaborative multicenter case-control study, we investigated the effect of factor V Leiden mutation, prothrombin gene mutation, and inherited deficiencies of protein C, protein S, and antithrombin on the risk of Budd-Chiari syndrome (BCS) and portal vein thrombosi

  5. Protein S testing in patients with protein S deficiency, factor V Leiden, and rivaroxaban by North American Specialized Coagulation Laboratories.

    Science.gov (United States)

    Smock, Kristi J; Plumhoff, Elizabeth A; Meijer, Piet; Hsu, Peihong; Zantek, Nicole D; Heikal, Nahla M; Van Cott, Elizabeth M

    2016-07-04

    In 2010-2012, the North American Specialized Coagulation Laboratory Association (NASCOLA) distributed 12 proficiency testing challenges to evaluate laboratory testing for protein S (PS). Results were analysed to assess the performance of PS activity, PS free antigen, and PS total antigen testing. Statistical analysis was performed on the numeric results and qualitative classification submitted for each method. There were 2,106 total results: 716 results from PS activity assays, 833 results from PS free antigen assays, and 557 results from PS total antigen assays. The three assay types performed well in the classification of five normal samples and nine abnormal samples, although certain PS activity methods were more likely to classify normal samples as abnormal and one PS total antigen assay was more likely to classify abnormal samples as normal. PS activity methods were affected by interfering substances such as heterozygous or homozygous factor V Leiden mutation (underestimation) and the anticoagulant drug rivaroxaban (overestimation). In conclusion, NASCOLA laboratories using a variety of PS assays performed well in the classification of clearly normal and abnormal samples. Laboratories performing PS activity assays should be aware of potential interferences in samples positive for FV Leiden or containing certain anticoagulant medications.

  6. Successful Living-Related Renal Allograft in a Recipient With Factor V Leiden Deficiency: A Case Report.

    Science.gov (United States)

    Florou, Evangelia; Koukoulaki, Maria; Theodoros, Theodoridis; Kalatzis, Vasileios; Vougas, Vasileios; Stamataki, Elissavet; Kokkinou, Vasiliki Christopoulou; Kostakis, Alkiviadis; Drakopoulos, Spiros

    2017-02-01

    Thrombophilia due to activated protein C resistance (Leiden mutation) is the most common inherited thrombophilic disorder with 5% incidence in whites. Renal transplant of these patients entails a risk of vascular thrombosis soon after the transplant; and acute rejection episodes and graft loss within the first year. We present a case of a successful living-related renal transplant in man with a recent history of repeat episodes of vascular access thrombosis attributed to inherited thrombophilia (heterozygosity for factor V mutation Q506 and homozygosity for mutation T677 for methylene-tetrahydrofolate reductase). Transplant recipient was administered anticoagulation therapy with low molecular weight heparin pre- and postoperatively. No thrombotic or hemorrhagic events occurred posttransplant. A high suspicion of thrombophilic disorders in patients with end-stage renal disease with vascular access thrombotic events should be screened further to prevent failure of a subsequent renal transplant. Inherited thrombophilic disorders may not exclude living-related kidney transplant provided that anticoagulation therapy is admin-istered perioperatively.

  7. Adherence to Surgical Antibiotic Prophylaxis Guidelines in New South Wales, Australia: Identifying Deficiencies and Regression Analysis of Contributing Factors.

    Science.gov (United States)

    Knox, Matthew C; Edye, Michael

    2016-04-01

    Surgical antibiotic prophylaxis is frequently reported in the literature to be suboptimal, a finding having both clinical and public health implications. This study aimed to calculate rates and patterns of adherence to guidelines at two sites and identify extrinsic contributing factors. A retrospective analysis was conducted over two 12-mo periods during 2013-2014 at the metropolitan Blacktown Hospital and regional Lismore Base Hospital, New South Wales, Australia. A group of 400 patients undergoing abdominal general surgery was selected via simple random sampling (n = 200 per site). Medical records were reviewed, and prophylactic antibiotic regimens were compared with the Australian guideline, Therapeutic Guidelines: Antibiotic (v. 14) with respect to drug choice, dosage, timing of administration, and duration of administration. The overall rate of adherence to the guidelines was 16.5% at Blacktown Hospital and 19.5% at Lismore Base Hospital. At each site, prophylaxis was administered to more than 95% of patients and was inappropriately withheld in 4%. Drug choice was the most frequent error type, specifically involving inappropriate omission of metronidazole and use of newer-generation cephalosporins. Errors in the timing of administration also were frequent, with prophylaxis typically occurring excessively early. Logistic regression identified emergency surgery as independently associated with prophylactic errors in both the Blacktown Hospital (p antibiotic prophylactic guidelines was poor at both the metropolitan and regional sites. Choice of antibiotic and timing of administration were identified as major error types. Consideration should be given to multidisciplinary involvement of anesthetists, implementation of focused interventions with an emphasis on emergency settings, and further research correlating antibiotic use with clinical significance.

  8. Apolipoprotein AI deficiency inhibits serum opacity factor activity against plasma high density lipoprotein via a stabilization mechanism.

    Science.gov (United States)

    Rosales, Corina; Patel, Niket; Gillard, Baiba K; Yelamanchili, Dedipya; Yang, Yaliu; Courtney, Harry S; Santos, Raul D; Gotto, Antonio M; Pownall, Henry J

    2015-04-14

    The reaction of Streptococcal serum opacity factor (SOF) against plasma high-density lipoproteins (HDL) produces a large cholesteryl ester-rich microemulsion (CERM), a smaller neo HDL that is apolipoprotein (apo) AI-poor, and lipid-free apo AI. SOF is active versus both human and mouse plasma HDL. In vivo injection of SOF into mice reduces plasma cholesterol ∼40% in 3 h while forming the same products observed in vitro, but at different ratios. Previous studies supported the hypothesis that labile apo AI is required for the SOF reaction vs HDL. Here we further tested that hypothesis by studies of SOF against HDL from apo AI-null mice. When injected into apo AI-null mice, SOF reduced plasma cholesterol ∼35% in 3 h. The reaction of SOF vs apo AI-null HDL in vitro produced a CERM and neo HDL, but no lipid-free apo. Moreover, according to the rate of CERM formation, the extent and rate of the SOF reaction versus apo AI-null mouse HDL were less than that against wild-type (WT) mouse HDL. Chaotropic perturbation studies using guanidine hydrochloride showed that apo AI-null HDL was more stable than WT HDL. Human apo AI added to apo AI-null HDL was quantitatively incorporated, giving reconstituted HDL. Both SOF and guanidine hydrochloride displaced apo AI from the reconstituted HDL. These results support the conclusion that apo AI-null HDL is more stable than WT HDL because it lacks apo AI, a labile protein that is readily displaced by physicochemical and biochemical perturbations. Thus, apo AI-null HDL is less SOF-reactive than WT HDL. The properties of apo AI-null HDL can be partially restored to those of WT HDL by the spontaneous incorporation of human apo AI. It remains to be determined what other HDL functions are affected by apo AI deletion.

  9. Vitamin D deficiency and low hemoglobin level as risk factors for severity of acute lower respiratory tract infections in Egyptian children: A case-control study

    Directory of Open Access Journals (Sweden)

    Abeer S. El Sakka

    2014-03-01

    Conclusion: Vitamin D deficiency was associated with severity of ALRTIs. Low hemoglobin level was more prevalent in those children. Improving the nutritional status in children by preventing vitamin D deficiency and low hemoglobin might influence the outcome of children with ALRTI.

  10. Iron deficiency

    DEFF Research Database (Denmark)

    Schou, Morten; Bosselmann, Helle; Gaborit, Freja

    2015-01-01

    BACKGROUND: Both iron deficiency (ID) and cardiovascular biomarkers are associated with a poor outcome in heart failure (HF). The relationship between different cardiovascular biomarkers and ID is unknown, and the true prevalence of ID in an outpatient HF clinic is probably overlooked. OBJECTIVES.......043). CONCLUSION: ID is frequent in an outpatient HF clinic. ID is not associated with cardiovascular biomarkers after adjustment for traditional confounders. Inflammation, but not neurohormonal activation is associated with ID in systolic HF. Further studies are needed to understand iron metabolism in elderly HF...

  11. Las tesis de los filósofos del siglo XIII que afirmaron la existencia del intelecto agente

    Directory of Open Access Journals (Sweden)

    Sellés, Juan Fernando

    2008-06-01

    Full Text Available In this article we review the statements of relevant philosophers of the XIIIeme century supporting the existence of the human agent intellect. Three groups can be distinguished: I The universal hylemorphism (Alexander of Hales, John de la Rochelle, Mathew of Acquasparta; II The synthesis between the augustinianism and the aristhotelism (Peter Hispanus, Saint Bonaventura, John Peckham; III The purified and advanced aristotelism (Albert the Great, Thomas Aquinas, Ramon Llull.

    En este trabajo se revisan las tesis de relevantes filósofos del s. XIII que defendieron la existencia del intelecto agente en el hombre. En el trabajo se dividen en tres grupos a los pensadores: I Los que se suelen encuadrar dentro del llamado hilemorfismo universal (Alejandro de Hales, Juan de la Rochela y Mateo de Acquasparta. II La síntesis entre el agustinismo y el aristotelismo (Pedro Hispano, San Buenaventura, Juan Peckham. III El aristotelismo depurado y proseguido (San Alberto Magno, Sto. Tomás de Aquino, Ramón Llull.

  12. Frontera y órdenes militares en la Edad Media castellano-leonesa (siglos XII-XIII

    Directory of Open Access Journals (Sweden)

    Carlos de AYALA MARTÍNEZ

    2009-12-01

    Full Text Available RESUMEN: El estudio presenta una breve panorámica del papel de las órdenes militares castellano-leonesas en la formación y consolidación de la frontera del reino con el Islam en los siglos XII y XIII. Ese papel tiene una triple dimensión –militar, colonizadora y estrictamente política– y sólo es posible constatar su eficacia a raíz del afianzamiento territorial e institucional de las órdenes militares, es decir, a partir del primer tercio del siglo XIII.ABSTRACT: The survey presents a brief view of the Castillian-Leonese military order’s role in the formation and consolidation of the kingdom’s border with the Islam in the twelfth and thirteenth centuries. This role has a triple dimension –military, settler and strictly political– and it is only possible to show its efficiency from the territorial and institutional strengthening of the military orders, that is, from the first third of the thirteenth century.

  13. An uncovered XIII century icon: particular use of organic pigments and gilding techniques highlighted by analytical methods.

    Science.gov (United States)

    Daveri, Alessia; Doherty, Brenda; Moretti, Patrizia; Grazia, Chiara; Romani, Aldo; Fiorin, Enrico; Brunetti, Brunetto Giovanni; Vagnini, Manuela

    2015-01-25

    The restoration of a panel painting depicting a Madonna and Child listed as an unknown Tuscan artist of the nineteenth century, permitted the hidden original version, a XIII century Medieval icon to be uncovered. It is discovery provided the opportunity for an extensive in situ campaign of non-invasive analytical investigations by portable imaging and spectroscopic techniques (infrared, X-ray fluorescence and diffraction, UV-Vis absorption and emission), followed by aimed micro-destructive investigations (Raman and SEM-EDS). This approach permitted characterization of the original ground and paint layers by complementary techniques. Furthermore, this protocol allowed supplementary particularities of great interest to be highlighted. Namely, numerous original gilding techniques have been accentuated in diverse areas and include the use of surrogate gold (disulphur tin), orpiment as a further false gold and an area with an original silver rich layer. Moreover, pigments including azurite mixed with indigo have been non-invasively identified. Micro-invasive analyses also allowed the diagnosis of organic colorants, namely, an animal anthraquinone lake, kermes and an unusual vegetal chalcone pigment, possibly safflower. The identification of the latter is extremely rare as a painting pigment and has been identified using an innovative adaption to surface enhanced Raman techniques on a cross-section. The resulting data contributes new hypotheses to the historic and artistic knowledge of materials and techniques utilized in XIII century icon paintings and ultimately provides scientific technical support of the recent restoration.

  14. An uncovered XIII century icon: Particular use of organic pigments and gilding techniques highlighted by analytical methods

    Science.gov (United States)

    Daveri, Alessia; Doherty, Brenda; Moretti, Patrizia; Grazia, Chiara; Romani, Aldo; Fiorin, Enrico; Brunetti, Brunetto Giovanni; Vagnini, Manuela

    2015-01-01

    The restoration of a panel painting depicting a Madonna and Child listed as an unknown Tuscan artist of the nineteenth century, permitted the hidden original version, a XIII century Medieval icon to be uncovered. It is discovery provided the opportunity for an extensive in situ campaign of non-invasive analytical investigations by portable imaging and spectroscopic techniques (infrared, X-ray fluorescence and diffraction, UV-Vis absorption and emission), followed by aimed micro-destructive investigations (Raman and SEM-EDS). This approach permitted characterization of the original ground and paint layers by complementary techniques. Furthermore, this protocol allowed supplementary particularities of great interest to be highlighted. Namely, numerous original gilding techniques have been accentuated in diverse areas and include the use of surrogate gold (disulphur tin), orpiment as a further false gold and an area with an original silver rich layer. Moreover, pigments including azurite mixed with indigo have been non-invasively identified. Micro-invasive analyses also allowed the diagnosis of organic colorants, namely, an animal anthraquinone lake, kermes and an unusual vegetal chalcone pigment, possibly safflower. The identification of the latter is extremely rare as a painting pigment and has been identified using an innovative adaption to surface enhanced Raman techniques on a cross-section. The resulting data contributes new hypotheses to the historic and artistic knowledge of materials and techniques utilized in XIII century icon paintings and ultimately provides scientific technical support of the recent restoration.

  15. Deregulation of Flk-1/vascular endothelial growth factor receptor-2 in fibroblast growth factor receptor-1-deficient vascular stem cell development.

    Science.gov (United States)

    Magnusson, Peetra; Rolny, Charlotte; Jakobsson, Lars; Wikner, Charlotte; Wu, Yan; Hicklin, Daniel J; Claesson-Welsh, Lena

    2004-03-15

    We have employed embryoid bodies derived from murine embryonal stem cells to study effects on vascular development induced by fibroblast growth factor (FGF)-2 and FGF receptor-1, in comparison to the established angiogenic factor vascular endothelial growth factor (VEGF)-A and its receptor VEGF receptor-2. Exogenous FGF-2 promoted formation of morphologically distinct, long slender vessels in the embryoid bodies, whereas VEGF-A-treated bodies displayed a compact plexus of capillaries. FGF-2 stimulation of embryonal stem cells under conditions where VEGF-A/VEGFR-2 function was blocked, led to formation of endothelial cell clusters, which failed to develop into vessels. FGFR-1(-/-) embryoid bodies responded to VEGF-A by establishment of the characteristic vascular plexus, but FGF-2 had no effect on vascular development in the absence of FGFR-1. The FGFR-1(-/-) embryoid bodies displayed considerably increased basal level of vessel formation, detected by immunohistochemical staining for platelet-endothelial cell adhesion molecule (PECAM)/CD31. This basal vascularization was blocked by neutralizing antibodies against VEGFR-2 or VEGF-A and biochemical analyses indicated changes in regulation of VEGFR-2 in the absence of FGFR-1 expression. We conclude that VEGF-A/VEGFR-2-dependent vessel formation occurs in the absence of FGF-2/FGFR-1, which, however, serve to modulate vascular development.

  16. Differential regulation of hepatic transcription factors in the Wistar rat offspring born to dams fed folic acid, vitamin B12 deficient diets and supplemented with omega-3 fatty acids.

    Science.gov (United States)

    Meher, Akshaya; Joshi, Asmita; Joshi, Sadhana

    2014-01-01

    Nutritional status of the mother is known to influence various metabolic adaptations required for optimal fetal development. These may be mediated by transcription factors like peroxisome proliferator activated receptors (PPARs), which are activated by long chain polyunsaturated fatty acids. The objective of the current study was to examine the expression of different hepatic transcription factors and the levels of global methylation in the liver of the offspring born to dams fed micronutrient deficient (folic acid and vitamin B12) diets and supplemented with omega-3 fatty acids. Female rats were divided into five groups (n = 8/group) as follows; control, folic acid deficient (FD), vitamin B12 deficient (BD) and omega-3 fatty acid supplemented groups (FDO and BDO). Diets were given starting from pre-conception and continued throughout pregnancy and lactation. Pups were dissected at the end of lactation. Liver tissues were removed; snap frozen and stored at -80°C. Maternal micronutrients deficiency resulted in lower (pacid (DHA) and arachidonic acid (ARA) as compared to the control group. Pup liver PPARα and PPARγ expression was lower (pfatty acids supplementation to this group normalized (pfatty acids supplementation to this group reduced (pfatty acid supplementation. Our novel findings suggest a role for omega-3 fatty acids in the one carbon cycle in influencing the hepatic expression of transcription factors in the offspring.

  17. Application of wide selected-ion monitoring data-independent acquisition to identify tomato fruit proteins regulated by the CUTIN DEFICIENT2 transcription factor.

    Science.gov (United States)

    Martin, Laetitia B B; Sherwood, Robert W; Nicklay, Joshua J; Yang, Yong; Muratore-Schroeder, Tara L; Anderson, Elizabeth T; Thannhauser, Theodore W; Rose, Jocelyn K C; Zhang, Sheng

    2016-08-01

    We describe here the use of label-free wide selected-ion monitoring data-independent acquisition (WiSIM-DIA) to identify proteins that are involved in the formation of tomato (Solanum lycopersicum) fruit cuticles and that are regulated by the transcription factor CUTIN DEFICIENT2 (CD2). A spectral library consisting of 11 753 unique peptides, corresponding to 2338 tomato protein groups, was used and the DIA analysis was performed at the MS1 level utilizing narrow mass windows for extraction with Skyline 2.6 software. We identified a total of 1140 proteins, 67 of which had expression levels that differed significantly between the cd2 tomato mutant and the wild-type cultivar M82. Differentially expressed proteins including a key protein involved in cutin biosynthesis, were selected for validation by target SRM/MRM and by Western blot analysis. In addition to confirming a role for CD2 in regulating cuticle formation, the results also revealed that CD2 influences pathways associated with cell wall biology, anthocyanin biosynthesis, plant development, and responses to stress, which complements findings of earlier RNA-Seq experiments. Our results provide new insights into molecular processes and aspects of fruit biology associated with CD2 function, and demonstrate that the WiSIM-DIA is an effective quantitative approach for global protein identifications.

  18. Pharmacogenomics of insulin-like growth factor-I generation during GH treatment in children with GH deficiency or Turner syndrome

    Science.gov (United States)

    Stevens, A; Clayton, P; Tatò, L; Yoo, H W; Rodriguez-Arnao, M D; Skorodok, J; Ambler, G R; Zignani, M; Zieschang, J; Della Corte, G; Destenaves, B; Champigneulle, A; Raelson, J; Chatelain, P

    2014-01-01

    Individual responses to growth hormone (GH) treatment are variable. Short-term generation of insulin-like growth factor-I (IGF-I) is recognized as a potential marker of sensitivity to GH treatment. This prospective, phase IV study used an integrated genomic analysis to identify markers associated with 1-month change in IGF-I (ΔIGF-I) following initiation of recombinant human (r-h)GH therapy in treatment-naïve children with GH deficiency (GHD) (n=166) or Turner syndrome (TS) (n=147). In both GHD and TS, polymorphisms in the cell-cycle regulator CDK4 were associated with 1-month ΔIGF-I (P<0.05). Baseline gene expression was also correlated with 1-month ΔIGF-I in both GHD and TS (r=0.3; P<0.01). In patients with low IGF-I responses, carriage of specific CDK4 alleles was associated with MAPK and glucocorticoid receptor signaling in GHD, and with p53 and Wnt signaling pathways in TS. Understanding the relationship between genomic markers and early changes in IGF-I may allow development of strategies to rapidly individualize r-hGH dose. PMID:23567489

  19. Potential Contribution of Iron Deficiency and Multiple Factors to Anemia Among 6- to 72-Month-Old Children in the Kokang Area of Myanmar.

    Science.gov (United States)

    Zhao, Ai; Gao, Hongchong; Li, Bo; Yu, Kai; Win, Naing Naing; Zhang, Yumei; Wang, Peiyu

    2015-10-01

    The prevalence of anemia among children in Myanmar has been reported to be among the highest in the world. This study was conducted to determine 1) the prevalence of anemia in preschool children and 2) risk factors associated with anemia. A total of 138 children aged from 6 to 72 months were recruited through cluster sampling from six villages in Kokang. Hemoglobin (Hb) concentration, blood trace elements, and anthropometric indicators were measured. Feces samples were collected to examine for the presence of ascarid eggs. The overall prevalence of anemia in children was 61.6%, including 10.9% with severe anemia. Meanwhile, high prevalence of stunting (40.0%), underweight (22.4%), wasting (6.3%), and small head circumference (6.7%) was found. Children with anemia were more prone to stunting. Children with severe anemia and moderate anemia had significantly lower blood iron and zinc levels than children without anemia (P water was positively associated with anemia (odds ratio [OR] = 6.368). This study demonstrated that anemia is an important public health problem among children from the Kokang area. Iron deficiency and drinking spring water may be the important causes of anemia among children. © The American Society of Tropical Medicine and Hygiene.

  20. Folate deficiency facilitates recruitment of upstream binding factor to hot spots of DNA double-strand breaks of rRNA genes and promotes its transcription.

    Science.gov (United States)

    Xie, Qiu; Li, Caihua; Song, Xiaozhen; Wu, Lihua; Jiang, Qian; Qiu, Zhiyong; Cao, Haiyan; Yu, Kaihui; Wan, Chunlei; Li, Jianting; Yang, Feng; Huang, Zebing; Niu, Bo; Jiang, Zhengwen; Zhang, Ting

    2016-12-06

    The biogenesis of ribosomes in vivo is an essential process for cellular functions. Transcription of ribosomal RNA (rRNA) genes is the rate-limiting step in ribosome biogenesis controlled by environmental conditions. Here, we investigated the role of folate antagonist on changes of DNA double-strand breaks (DSBs) landscape in mouse embryonic stem cells. A significant DSB enhancement was detected in the genome of these cells and a large majority of these DSBs were found in rRNA genes. Furthermore, spontaneous DSBs in cells under folate deficiency conditions were located exclusively within the rRNA gene units, representing a H3K4me1 hallmark. Enrichment H3K4me1 at the hot spots of DSB regions enhanced the recruitment of upstream binding factor (UBF) to rRNA genes, resulting in the increment of rRNA genes transcription. Supplement of folate resulted in a restored UBF binding across DNA breakage sites of rRNA genes, and normal rRNA gene transcription. In samples from neural tube defects (NTDs) with low folate level, up-regulation of rRNA gene transcription was observed, along with aberrant UBF level. Our results present a new view by which alterations in folate levels affects DNA breakage through epigenetic control leading to the regulation of rRNA gene transcription during the early stage of development.

  1. Potential Contribution of Iron Deficiency and Multiple Factors to Anemia among 6- to 72-Month-Old Children in the Kokang Area of Myanmar

    Science.gov (United States)

    Zhao, Ai; Gao, Hongchong; Li, Bo; Yu, Kai; Win, Naing Naing; Zhang, Yumei; Wang, Peiyu

    2015-01-01

    The prevalence of anemia among children in Myanmar has been reported to be among the highest in the world. This study was conducted to determine 1) the prevalence of anemia in preschool children and 2) risk factors associated with anemia. A total of 138 children aged from 6 to 72 months were recruited through cluster sampling from six villages in Kokang. Hemoglobin (Hb) concentration, blood trace elements, and anthropometric indicators were measured. Feces samples were collected to examine for the presence of ascarid eggs. The overall prevalence of anemia in children was 61.6%, including 10.9% with severe anemia. Meanwhile, high prevalence of stunting (40.0%), underweight (22.4%), wasting (6.3%), and small head circumference (6.7%) was found. Children with anemia were more prone to stunting. Children with severe anemia and moderate anemia had significantly lower blood iron and zinc levels than children without anemia (P anemia. Drinking spring water was positively associated with anemia (odds ratio [OR] = 6.368). This study demonstrated that anemia is an important public health problem among children from the Kokang area. Iron deficiency and drinking spring water may be the important causes of anemia among children. PMID:26195457

  2. Reduced expression of brain-derived neurotrophic factor in mice deficient for pituitary adenylate cyclase activating polypeptide type-I-receptor.

    Science.gov (United States)

    Zink, Mathias; Otto, Christiane; Zörner, Björn; Zacher, Christiane; Schütz, Günther; Henn, Fritz A; Gass, Peter

    2004-04-22

    In vitro pituitary adenylate cyclase activating polypeptide (PACAP) induces the expression of brain-derived neurotrophic factor (BDNF) via its specific receptor PAC1. Since BDNF has been implicated in learning paradigms and mice lacking functional PAC1 have deficits in hippocampus-dependent associative learning, we investigated whether PAC1 mutants show alterations in hippocampal expression of BDNF and its receptor TrkB. Semi-quantitative in situ-hybridization using exon-specific BDNF-probes revealed significantly reduced expression of the exon-III and exon-V-specific transcripts within the hippocampal CA3 region in PAC1-deficient mice. A similar trend was observed for the exon-I-specific transcript. The expression of the exon-III-specific transcript was also reduced within the dentate gyrus, while Trk B-expression did not differ between genotypes. Our data demonstrate that even in vivo PAC1-mediated signaling seems to play a pivotal role for the transcriptional regulation of BDNF.

  3. A future for soil ecology? Connecting the system levels: moving from genomes to ecosystems; opening lecture to the XIII ICSZ "Biodiversity of soil organisms and ecosystem functioning"

    NARCIS (Netherlands)

    Eijsacker, H.J.P.

    2001-01-01

    As an introduction to the XIII ICSZ "Biodiversity of soil organisms and ecosystem functioning" the question is raised what contribution soil ecology has made to general ecology. Although the appearance of soil ecological papers in general ecological journals is limited, soil ecologists have had a ma

  4. Detection and identification of the heterogeneous novel subgroup 16SrXIII-(A/I)I phytoplasma associated with strawberry green petal disease and Mexican periwinkle virescence.

    Science.gov (United States)

    Pérez-López, Edel; Dumonceaux, Tim J

    2016-11-01

    Phytoplasmas (species of the genus 'CandidatusPhytoplasma') are insect-vectored phytopathogenic bacteria associated with economically and ecologically important crop diseases. Strawberry production represents an important part of agricultural activity in Mexico and elsewhere, and infection of plants with phytoplasma renders the fruit inedible by altering plant development, resulting in virescence and phyllody. In this study we examined samples taken from four strawberry plants showing symptoms associated with strawberry green petal disease and from two periwinkle plants showing virescence, sampled in different areas of Mexico. Analysis of the 16S rRNA-encoding sequences showed that the plants were infected with a phytoplasma previously identified as Mexican periwinkle virescence (MPV; 16SrXIII). Examination of bacterial sequences from these samples revealed that two distinct 16S rRNA gene sequences were present in each sample along with a single chaperonin-60 (cpn60) sequence and a single rpoB sequence, suggesting that this strain displays 16S rRNA gene sequence heterogeneity. Two distinct rrn operons, identified with subgroup 16SrXIII-A and the newly described subgroup 16SrXIII-I, were identified from the six samples analyzed, delineating the novel subgroup 16SrXIII-(A/I)I, following the nomenclature proposed for heterogeneous subgroups.

  5. Leptin stimulates fibroblast growth factor 23 expression in bone and suppresses renal 1alpha,25-dihydroxyvitamin D3 synthesis in leptin-deficient mice.

    Science.gov (United States)

    Tsuji, Kiyomi; Maeda, Toyonobu; Kawane, Tetsuya; Matsunuma, Ayako; Horiuchi, Noboru

    2010-08-01

    Leptin is the LEP (ob) gene product secreted by adipocytes. We previously reported that leptin decreases renal expression of the 25-hydroxyvitamin D(3) 1alpha-hydroxylase (CYP27B1) gene through the leptin receptor (ObRb) by indirectly acting on the proximal tubules. This study focused on bone-derived fibroblast growth factor 23 (FGF-23) as a mediator of the influence of leptin on renal 1alpha-hydroxylase mRNA expression in leptin-deficient ob/ob mice. Exposure to leptin (200 ng/mL) for 24 hours stimulated FGF-23 expression by primary cultured rat osteoblasts. Administration of leptin (4 mg/kg i.p. at 12-hour intervals for 2 days) to ob/ob mice markedly increased the serum FGF-23 concentration while significantly reducing the serum levels of calcium, phosphate, and 1alpha,25-dihydroxyvitamin D(3) [1,25(OH)(2)D(3)]. Administration of FGF-23 (5 microg i.p. at 12-hour intervals for 2 days) to ob/ob mice suppressed renal 1alpha-hydroxylase mRNA expression. The main site of FGF-23 mRNA expression was the bone, and leptin markedly increased the FGF-23 mRNA level in ob/ob mice. In addition, leptin significantly reduced 1alpha-hydroxylase and sodium-phosphate cotransporters (NaP(i)-IIa and NaP(i)-IIc) mRNA levels but did not affect Klotho mRNA expression in the kidneys of ob/ob mice. Furthermore, the serum FGF-23 level and renal expression of 1alpha-hydroxylase mRNA were not influenced by administration of leptin to leptin receptor-deficient (db/db) mice. These results indicate that leptin directly stimulates FGF-23 synthesis by bone cells in ob/ob mice, suggesting that inhibition of renal 1,25(OH)(2)D(3) synthesis in these mice is at least partly due to elevated bone production of FGF-23.

  6. Environmental risk factors for iron deficiency anemia in children 12-24 months old in the area of Thessalia in Greece.

    Science.gov (United States)

    Tympa-Psirropoulou, E; Vagenas, C; Dafni, O; Matala, A; Skopouli, F

    2008-01-01

    Iron deficiency anemia (IDA) is a common problem all over the world, which attacks mainly pregnant women, infants and children. The aim of the study was to estimate the prevalence of IDA in children 12-24 months old in a specific area of Thessalia, located in the central part of Greece, and to identify the environmental risk factors associated with it. In the first part of this cross-sectional and case-control study, the hemoglobin (Hb) levels of 938 children were estimated by a mobile photometer analyzer. In the second part of the study, children with Hbzinc protoporphyrin, serum iron, serum ferritin, transferring saturation, total iron binding capacity and Hb electrophoresis. Finally 75 children (34 boys, 41 girls, mean age 17.51+/-3.5 months), who were found with IDA, constituted the case group while 75 healthy children constituted the control group. The prevalence of IDA in the region was 7.99%. At the same time, a number of children with stigma of bthalassaemia (2.13%) was discovered, something that had escaped identification. There were no differences due to the method of determination (mobile or laboratory) in the values of Hb between the two groups. Significant differences were recorded (pwater and sewage system (pworld, it still consists a public health problem. The mobile method for Hb estimation should be introduced in Greece since its reliability to detect IDA has been, once more, confirmed. The application of simple questionnaires for the detection of the environmental IDA risk factors could help in the prognosis and prevention of anemia. Further improvement of the IDA status in Greece could be achieved through the dissemination of information about iron rich foods, the amelioration of environmental conditions and the application of reliable, easy to use and cheap methods for Hb estimation.

  7. Genotrim, a DNA-customized nutrigenomic product, targets genetic factors of obesity: hypothesizing a dopamine-glucose correlation demonstrating reward deficiency syndrome (RDS).

    Science.gov (United States)

    Blum, Kenneth; Chen, Thomas J H; Meshkin, Brian; Downs, B William; Gordon, Cory A; Blum, Seth; Mangucci, Julie F; Braverman, Eric R; Arcuri, Vanessa; Deutsch, Roger; Pons, Manuel-Martinez-

    2007-01-01

    Obesity is the second largest cause of preventable death in the United States. Historically, obesity was considered a behavioral problem that could be simply addressed with behavioral modifications in diet and exercise. As scientific advancements have demonstrated in other neurological healthcare conditions such as alcoholism, there are important biological and genetic components that limit the efficacy of behavioral adjustments alone. In light of data suggesting frequent co-morbidities to obesity, including diabetes mellitus, atherosclerosis, osteoporosis, and potentially others, we hypothesize that the biologic and genetic factors, synergistically with behavioral modifications, must be addressed to adequately treat this disease. We hypothesize that one such genetic factor that influences behavior and thus obesity is a predisposition to glucose craving and the overall effect of dopaminergic activity in the reward center of the brain. This defect drives individuals to engage in activities of behavioral excess, which will increase brain dopamine function, for which we have created the term reward deficiency syndrome (RDS) to categorize such biological influences on behavior. Consuming large quantities of alcohol or carbohydrates (carbohydrate bingeing) stimulates the brain's production of and utilization of dopamine. So too does the intake of crack/cocaine and the abuse of nicotine. We are proposing that a novel approach to nutritional supplementation may be required to target the RDS role in obesity. In this regard, Genotrim, a DNA based customized nutraceutical has been designed and is currently under investigation in several clinical studies. This is the first hypothesis paper whereby this new paradigm shift in thinking about obesity is presented.

  8. Zinc-dependent lysosomal enlargement in TRPML1-deficient cells involves MTF-1 transcription factor and ZnT4 (Slc30a4) transporter.

    Science.gov (United States)

    Kukic, Ira; Lee, Jeffrey K; Coblentz, Jessica; Kelleher, Shannon L; Kiselyov, Kirill

    2013-04-15

    Zinc is critical for a multitude of cellular processes, including gene expression, secretion and enzymatic activities. Cellular zinc is controlled by zinc-chelating proteins and by zinc transporters. The recent identification of zinc permeability of the lysosomal ion channel TRPML1 (transient receptor potential mucolipin 1), and the evidence of abnormal zinc levels in cells deficient in TRPML1, suggested a role for TRPML1 in zinc transport. In the present study we provide new evidence for such a role and identify additional cellular components responsible for it. In agreement with the previously published data, an acute siRNA (small interfering RNA)-driven TRPML1 KD (knockdown) leads to the build-up of large cytoplasmic vesicles positive for LysoTracker™ and zinc staining, when cells are exposed to high concentrations of zinc. We now show that lysosomal enlargement and zinc build-up in TRPML1-KD cells exposed to zinc are ameliorated by KD of the zinc-sensitive transcription factor MTF-1 (metal-regulatory-element-binding transcription factor-1) or the zinc transporter ZnT4. TRPML1 KD is associated with a build-up of cytoplasmic zinc and with enhanced transcriptional response of mRNA for MT2a (metallothionein 2a). TRPML1 KD did not suppress lysosomal secretion, but it did delay zinc leak from the lysosomes into the cytoplasm. These results underscore a role for TRPML1 in zinc metabolism. Furthermore, they suggest that TRPML1 works in concert with ZnT4 to regulate zinc translocation between the cytoplasm and lysosomes.

  9. Hyperhomocysteinemia-Induced Monocyte Chemoattractant Protein-1 Promoter DNA Methylation by Nuclear Factor-κB/DNA Methyltransferase 1 in Apolipoprotein E-Deficient Mice.

    Science.gov (United States)

    Wang, Ju; Jiang, Yideng; Yang, Anning; Sun, Weiwei; Ma, Changjian; Ma, Shengchao; Gong, Huihui; Shi, Yingkang; Wei, Jun

    2013-04-01

    Hyperhomocysteinemia is considered to be a significant risk factor in atherosclerosis and plays an important role in it. The purpose of this study was to determine the molecular mechanism of blood monocyte chemoattractant protein-1 (MCP-1) promoter DNA hypomethylation in the formation of atherosclerosis induced by hyperhomocysteinemia, and to explore the effect of nuclear factor-κB (NF-κB)/DNA methyltransferase 1 (DNMT1) in this mechanism. The atherosclerotic effect of MCP-1 in apolipoprotein E-deficient (ApoE(-/-)) and wild-type C57BL/6J mice was evaluated using atherosclerotic lesion area; serum NF-κB, MCP-1, and DNMT1 levels; and MCP-1 promoter DNA methylation expression. In vitro, the mechanism responsible for the effect of NF-κB/DNMT1 on foam cells was investigated by measuring NF-κB and DNMT1 levels to determine whether NF-κB/DNMT1 had an effect on gene expression. Compared with the control group, atherosclerotic lesions in ApoE(-/-) mice fed a high methionine diet significantly increased, as did the expression of MCP-1. In vitro study showed that pyrrolidine dithiocarbamate treatment down-regulated levels of NF-κB and raised DNMT1 concentrations, confirming the effect of NF-κB/DNMT1 in the MCP-1 promoter DNA methylation process. In conclusion, our results suggest that through NF-κB/DNMT1, MCP-1 promoter DNA hypomethylation may play a key role in formation of atherosclerosis under hyperhomocysteinemia.

  10. Evolution and treatment of vitamin B12 deficiency as a risk factor for (cognitive and functional) neurodegenerative diseases in institutionalized elderly = Evolución y tratamiento de la deficiencia de vitamina B12 como factor de riesgo de enfermedades neurodegenerativas (cognitivas y funcionales) en las personas mayores institucionalizadas.

    OpenAIRE

    Albers, Ulrike

    2012-01-01

    Prevalence of vitamin B12 deficiency is very common in elderly people and can reach values as high as 40.5% of the population. It can be the result of the interaction among several factors. Vitamin B12 deficiencies have been associated with neurological, cognitive deterioration, haematological abnormalities and cardiovascular diseases that have an important influence on the health of the elderly and their quality of life. It is necessary to approach the problems arisen from the lack of data r...

  11. Estratégia anestesiológica para cesariana em paciente portadora de deficiência de fator XI: relato de caso Estrategia anestesiológica para cesárea en paciente portadora de discapacidad de Factor XI: relato de caso Anesthetic strategy for cesarean section in a patient with factor XI deficiency: case report

    Directory of Open Access Journals (Sweden)

    Norma Sueli P Módolo

    2010-04-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A deficiência do fator XI é uma doença hematológica rara na população. A hemofilia C (deficiência do fator XI ocorre em ambos os sexos e normalmente não apresenta qualquer sintomatologia, podendo manifestar-se apenas como hemorragia pós-cirúrgica. É uma doença autossômica recessiva, homozigótica ou heterozigótica, e sua gravidade depende dos níveis de fator XI. O objetivo desse relato foi apresentar a estratégia anestésica em paciente portadora de hemofilia C. RELATO DO CASO: Paciente com 32 anos, gesta I/para 0, 39 semanas de gestação programada para cesariana eletiva. Paciente portadora de deficiência de fator XI. Exame clínico e laboratorial sem alterações. Conforme orientação do hematologista, no dia da cesárea a paciente usou prometazina 25 mg; hidrocortisona 500 mg, devido a reações transfusionais prévias, e plasma 10 mL-1.kg-1 num total de 700 mL. Após 2 horas foi submetida ao bloqueio subaracnóideo sob monitorização de rotina. Hidratação com RL 2000 mL. Procedimento anestésico-cirúrgico sem intercorrências. A paciente evoluiu no pós-operatório sem intercorrências, sendo que no 3º DPO fez uso de plasma fresco congelado (PFC 10.mL-1.kg-1 com o objetivo de evitar sangramento pós cirúrgico tardio. CONCLUSÕES: O objetivo do caso foi apresentar o protocolo anestésico para pacientes portadores de hemofilia C e alertar para a necessidade de investigação em caso de antecedente de sangramento pós-operatório, quando um estudo da coagulação deve ser realizado antes de qualquer procedimento invasivo e, se um TTPA prolongado for encontrado, torna-se imperativo pesquisar a deficiência desse fator.JUSTIFICATIVA Y OBJETIVOS: La discapacidad del factor XI es una enfermedad hematológica rara en la población. La hemofilia C (discapacidad del factor XI, ocurre en los dos sexos y normalmente no presenta ninguna sintomatología, y se puede manifestar apenas como hemorragia post

  12. Vitamin D deficiency in adolescents

    OpenAIRE

    Ashraf T Soliman; Vincenzo De Sanctis; Rania Elalaily; Said Bedair; Islam Kassem

    2014-01-01

    The prevalence of severe vitamin D deficiency (VDD) in adolescents is variable but considerably high in many countries, especially in Middle-east and Southeast Asia. Different factors attribute to this deficiency including lack of sunlight exposure due to cultural dress codes and veiling or due to pigmented skin, and less time spent outdoors, because of hot weather, and lower vitamin D intake. A potent adaptation process significantly modifies the clinical presentation and therefore clinical ...

  13. Comorbidity and cardiovascular risk factors in adult GH deficiency following treatment for Cushing's disease or non-functioning pituitary adenomas during childhood

    DEFF Research Database (Denmark)

    Ragnarsson, Oskar; Höybye, Charlotte; Jönsson, Peter J;

    2012-01-01

    Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood.......Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood....

  14. Of decentralization of public power Ukrainian land that belonged to Lithuanian (XIII – the early XVII century

    Directory of Open Access Journals (Sweden)

    C. V. Manuilova

    2016-06-01

    Full Text Available A comprehensive crisis in Ukraine and continued military confrontation in the Donbass demonstrated the urgent need to establish effective governance, which would imply decentralization of public power. Note that in implementing the decentralization of power in Ukraine insists the International Monetary Fund; United Nations Development Program; the transfer of authority to the field and decentralization of power in Ukraine is one of the points of the Minsk agreements and obligations of Ukraine to the EU. The article deals with the Ukrainian lands topical issue features the decentralization of public power in the XIII - the beginning of XVII century. The importance of the topic due to the need to study the historical experience of the implementation of decentralization. It was, emphasized that the success of the reforms depends largely because of the historical experience and features of the decentralization of public power in the past. Characterized by the development of local government in the Ukrainian lands was part of the Lithuanian state. The purpose of the article is to clarify the characteristics of decentralization of public authority on Ukrainian lands were part of the Lithuanian state during the XVII century XIII. To address this goal, outline decentralization of public power in the state; analyze, competence of local government in the Ukrainian lands that belonged to the Lith uanian State; determine how close to the power of the people. The level of decentralization of public power in the Grand Duchy of Lithuania in the XIII - the beginning of XVII century was high. It was, found that Lithuania had not established a centralized state. It is, noted that the Board of the nobility limited the princely power. The effect of delegated deputies from different parts of the Lithuanian statehood solutions nobility Council.Clarified the facts that confirm the existence of decentralization of public power in Lithuania: the functioning of local

  15. Impaired glucose metabolism is a risk factor for increased thyroid volume and nodule prevalence in a mild-to-moderate iodine deficient area.

    Science.gov (United States)

    Anil, Cuneyd; Akkurt, Aysen; Ayturk, Semra; Kut, Altug; Gursoy, Alptekin

    2013-07-01

    Insulin resistance (IR) is a key factor involved in the pathogenesis of impaired glucose metabolism. IR is associated with increased thyroid volume and nodule prevalence in patients with metabolic syndrome. Data on the association of thyroid morphology and abnormal glucose metabolism are limited. This prospective study was carried out to evaluate thyroid volume and nodule prevalence in patients with pre-diabetes and type 2 diabetes mellitus (DM) in a mild-to-moderate iodine deficient area. Data were gathered on all newly diagnosed patients with pre-diabetes and type 2 diabetes mellitus between May 2008 and February 2010. 156 patients with pre-diabetes and 123 patients with type 2 DM were randomly matched for age, gender, and smoking habits with 114 subjects with normal glucose metabolism. Serum thyroid-stimulating hormone (TSH) and thyroid ultrasonography was performed in all participants. Mean TSH level in the diabetes group (1.9±0.9 mIU/L) was higher than in the control group (1.4±0.8 mIU/L) and the pre-diabetes group (1.5±0.8 mIU/L) (P<0.0001 for both). Mean thyroid volume was higher in the pre-diabetes (18.2±9.2mL) and diabetes (20.0±8.2mL) groups than in controls (11.4±3.8mL) (P<0.0001 for both). Percentage of patients with thyroid nodules was also higher in the pre-diabetes (51.3%) and diabetes groups (61.8%) than in controls (23.7%) (P<0.0001 for both). The results suggest that patients with impaired glucose metabolism have significantly increased thyroid volume and nodule prevalence. Copyright © 2013 Elsevier Inc. All rights reserved.

  16. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... at highest risk for iron-deficiency anemia. Outlook Doctors usually can successfully treat iron-deficiency anemia. Treatment ... poor skin tone, dizziness, and depression. After her doctor diagnosed her with iron-deficiency anemia, Susan got ...

  17. Patho-epidemiological study on Genotype-XIII Newcastle disease virus infection in commercial vaccinated layer farms

    Directory of Open Access Journals (Sweden)

    J. H. Khorajiya

    2015-03-01

    Full Text Available Aim: The present research work was carried out to study the patho-epidemiological aspects of Genotype-XIII Newcastle disease virus (NDV infection in commercial layer in and around Anand, Gujarat. As the outbreaks have reported in vaccinated flocks, it was felt necessary to study the disease with respect to its changing pathogenicity and relevant aspects. Materials and Methods: The study comprised of patho-epidemiology of Newcastle disease (ND by information collected from different layer farms suffering from the disease in relation to incidence pattern and mortality, duration of mortality, susceptible age, and loss due to production performance. Clinical signs were recorded based on observations. During postmortem, gross lesions were also recorded. For histopathological examination visceral organs according to lesions were collected in 10% formalin and processed slide stained by hematoxylin and eosin for microscopic examination. Cultivation of virus was done in embryonated specific pathogen-free (SPF eggs of 9-11 days and isolation of virus was done for haemagglutination (HA and haemagglutination inhibition (HI test and to identify pathotype of virus by intracerebral pathogenicity index (ICPI test to determine the virulence of virus. The Genotype-XIII NDV was confirmed by F gene sequence and whole genome sequence. Results: During the study mortality due to ND was recorded in 13 layer flocks in spite of routine vaccination, which usually contain Genotype-II strain of virus. The mortality was observed as high as above 50% with an average of 21.21%. The susceptible age for disease was found to be 6-14 weeks. The duration of mortality observed was 23 days. The disease resulted in a significant reduction in body weight, feed intake and drop in egg production. Majority of the outbreaks appeared during extremely hot months of April to June. Greenish diarrhoea was frequently seen in birds that survived early in infection. Mortality continued for 2

  18. Servus pauperum Christi: los servicios de los Hospitalarios a los peregrinos en el Oriente latino, siglos XI-XIII

    Directory of Open Access Journals (Sweden)

    Judith Bronstein

    2013-12-01

    Full Text Available Dos rasgos principales caracterizan el renacimiento religioso del siglo XI: un crecimien-to de la peregrinación a los lugares santos y un nuevo énfasis en la actividad apostólica y evan-gelizadora. La combinación de los dos lleva a la creación de una de las instituciones religiosas más originales del Este: el Hospital de San Juan, establecido poco antes de 1071 como hospicio para los peregrinos pobres y enfermos que llegaban a Jerusalén. Este artículo analiza sus orígenes y actividades en cuanto organización internacional establecida para el servicio de los peregrinos y de los pobres en el Oriente Latino desde el siglo XI al siglo XIII

  19. Las armas de los Limia y sus derivaciones (siglos XIII-XV

    Directory of Open Access Journals (Sweden)

    Eduardo PARDO DE GUEVARA Y VALDÉS

    2011-07-01

    Full Text Available En los primeros tiempos del fenómeno emblemático-heráldico, la adopción de unas armerías no respondía a un proceso reflexivo, tal y como ahora parecería natural, sino a unos impulsos espontáneos, en los que predominaba el simple gusto o capricho, cuando no la directa imitación o aceptación de modelos ya existentes, con independencia de la significación o valor social que se les reconociera. Un buen testimonio de ello lo ofrecen las armas traídas ya antes de mediar el siglo XIII por el linaje renombrado Limia, cuyo análisis ofrece lecturas interesantes para comprender el sentido y alcance de estos procedimientos habituales. Como se trata aquí, los palos y palados de los Limia fueron traídos después por los Valcárcel y, más tarde, por un pequeño número de linajes de ellos derivados, aunque las modificaciones introducidas parecen ocultar este origen: las varillas de los Varela, las correas de los Isorna, las lanzas parlantes de los Lanzós -o Lanzones- o las estacas de los Quiroga, así como la losa de los Losada y las fórmulas fusionadas de palos y calderas, cuyo inicio pudo estar en los Biedma y que no tardaría en identificar también al grupo de los Taboada y Noguerol.Aux premiers temps du phénomène emblématique-héraldique, l’adoption d'armoiries répondait moins à un processus réfléchi comme on s’y attendrait aujourd’hui, qu’à des élans spontanés guidés par le simple plaisir ou le caprice, ou même par l’imitation ou l’adoption de quelques modèles déjà existants, indépendamment de la signification ou de la valeur sociale qu’on pourrait leur reconnaître. Tel est le cas des armes portées dans la première moitié du XIIIe siècle par le lignage des Limia, dont l’analyse permet de  comprendre le sens et la portée de ces procédés. Comme il sera dit dans cette étude, les pals et palés des Limia ont été ensuite portés par les Valcárcel et, plus tard, par un petit nombre de lignages

  20. International Retrospective Chart Review of Treatment Patterns in Severe Familial Mediterranean Fever, Tumor Necrosis Factor Receptor-Associated Periodic Syndrome, and Mevalonate Kinase Deficiency/Hyperimmunoglobulinemia D Syndrome.

    Science.gov (United States)

    Ozen, Seza; Kuemmerle-Deschner, Jasmin B; Cimaz, Rolando; Livneh, Avi; Quartier, Pierre; Kone-Paut, Isabelle; Zeft, Andrew; Spalding, Steve; Gul, Ahmet; Hentgen, Veronique; Savic, Sinisa; Foeldvari, Ivan; Frenkel, Joost; Cantarini, Luca; Patel, Dony; Weiss, Jeffrey; Marinsek, Nina; Degun, Ravi; Lomax, Kathleen G; Lachmann, Helen J

    2017-04-01

    Periodic fever syndrome (PFS) conditions are characterized by recurrent attacks of fever and localized inflammation. This study examined the diagnostic pathway and treatments at tertiary centers for familial Mediterranean fever (FMF), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), and mevalonate kinase deficiency (MKD)/hyperimmunoglobulinemia D syndrome (HIDS). PFS specialists at medical centers in the US, the European Union, and the eastern Mediterranean participated in a retrospective chart review, providing de-identified data in an electronic case report form. Patients were treated between 2008 and 2012, with at least 1 year of followup; all had clinical and/or genetically proven disease and were on/eligible for biologic treatment. A total of 134 patients were analyzed: FMF (n = 49), TRAPS (n = 47), and MKD/HIDS (n = 38). Fever was commonly reported as severe across all indications. Other frequently reported severe symptoms were serositis for FMF patients and elevated acute-phase reactants and gastrointestinal upset for TRAPS and MKD/HIDS. A long delay from disease onset to diagnosis was seen within TRAPS and MKD/HIDS (5.8 and 7.1 years, respectively) compared to a 1.8-year delay in FMF patients. An equal proportion of TRAPS patients first received anti-interleukin-1 (anti-IL-1) and anti-tumor necrosis factor (anti-TNF) biologic agents, whereas IL-1 blockade was the main choice for FMF patients resistant to colchicine and MKD/HIDS patients. For TRAPS patients, treatment with anakinra versus anti-TNF treatments as first biologic agent resulted in significantly higher clinical and biochemical responses (P = 0.03 and P < 0.01, respectively). No significant differences in responses were observed between biologic agents among other cohorts. Referral patterns and diagnostic delays highlight the need for greater awareness and improved diagnostics for PFS. This real-world treatment assessment supports the need for further

  1. Analysis of Pathogenic Factors in Children with Iron Deficiency Anemia%小儿营养性缺铁性贫血致病因素的分析

    Institute of Scientific and Technical Information of China (English)

    张爱莲

    2014-01-01

    Children with iron deficiency anemia, is due to the body iron deficiency leads to decrease in hemoglobin synthesis, to 6~36 months of age group. Due to the improvement of living standards, a variety of nutritional deficiency have been significantly reduced, but the iron deficiency anemia is common, have serious adverse ef ects on health, we must actively control. Therefore, in our hospital found that children with iron deficiency anemia report is as fol ows.%小儿营养性缺铁性贫血,是由于体内铁缺乏导致血红蛋白合成减少所致,以6~36个月为高发年龄组。由于生活水平的提高,各种营养缺乏症都已明显减少,但是缺铁性贫血仍很常见,对健康有严重不良影响,必须积极防治。故此,对在我院就诊发现小儿营养性缺铁性贫血情况报告如下。

  2. Dietary iodine deficiency in the Gippsland region of Victoria, Australia

    OpenAIRE

    Rahman, Mohammad Ashequr

    2017-01-01

    Background Iodine is an essential micronutrient for the production of thyroid hormones and normal neurodevelopment. A deficiency in iodine causes a number of defects collectively known as Iodine Deficiency Disorder (IDD). Even mild iodine deficiency in pregnancy is a risk factor for babies as it may result in impaired intellectual development; this is the most serious consequence of mild to moderate dietary iodine deficiency. Australia overall is iodine deficient. However, in the Nationa...

  3. Real-time PCR demonstrates Ancylostoma duodenale is a key factor in the etiology of severe anemia and iron deficiency in Malawian pre-school children.

    Science.gov (United States)

    Jonker, Femkje A M; Calis, Job C J; Phiri, Kamija; Brienen, Eric A T; Khoffi, Harriet; Brabin, Bernard J; Verweij, Jaco J; van Hensbroek, Michael Boele; van Lieshout, Lisette

    2012-01-01

    Hookworm infections are an important cause of (severe) anemia and iron deficiency in children in the tropics. Type of hookworm species (Ancylostoma duodenale or Necator americanus) and infection load are considered associated with disease burden, although these parameters are rarely assessed due to limitations of currently used diagnostic methods. Using multiplex real-time PCR, we evaluated hookworm species-specific prevalence, infection load and their contribution towards severe anemia and iron deficiency in pre-school children in Malawi. A. duodenale and N. americanus DNA loads were determined in 830 fecal samples of pre-school children participating in a case control study investigating severe anemia. Using multiplex real-time PCR, hookworm infections were found in 34.1% of the severely anemic cases and in 27.0% of the non-severely anemic controls (pIron deficiency (assessed through bone marrow examination) was positively associated with intensity of A. duodenale infection (adjusted odds ratio: 3.63 (95%CI 1.18-11.20); 16.98 (95%CI 3.88-74.35) and 44.91 (95%CI 5.23-385.77) for low, moderate and high load respectively). This is the first report assessing the association of hookworm load and species differentiation with severe anemia and bone marrow iron deficiency. By revealing a much higher than expected prevalence of A. duodenale and its significant and load-dependent association with severe anemia and iron deficiency in pre-school children in Malawi, we demonstrated the need for quantitative and species-specific screening of hookworm infections. Multiplex real-time PCR is a powerful diagnostic tool for public health research to combat (severe) anemia and iron deficiency in children living in resource poor settings.

  4. Smac Mimetic Bypasses Apoptosis Resistance in FADD- or Caspase-8-Deficient Cells by Priming for Tumor Necrosis Factor α-Induced Necroptosis

    Directory of Open Access Journals (Sweden)

    Bram Laukens

    2011-10-01

    Full Text Available Searching for new strategies to bypass apoptosis resistance, we investigated the potential of the Smac mimetic BV6 in Jurkat leukemia cells deficient in key molecules of the death receptor pathway. Here, we demonstrate for the first time that Smac mimetic primes apoptosis-resistant, FADD- or caspase-8-deficient leukemia cells for TNFα-induced necroptosis in a synergistic manner. In contrast to TNFα, Smac mimetic significantly enhances CD95-induced apoptosis in wild-type but not in FADD-deficient cells. Interestingly, Smac mimetic- and TNFα-mediated cell death occurs without characteristic features of apoptosis (i.e., caspase activation, DNA fragmentation in FADD-deficient cells. By comparison, Smac mimetic and TNFα trigger activation of caspase-8, -9, and -3 and DNA fragmentation in wild-type cells. Consistently, the caspase inhibitor zVAD.fmk fails to block Smac mimetic- and TNFα-triggered cell death in FADD- or caspase-8-deficient cells, while it confers protection in wild-type cells. By comparison, necrostatin-1, an RIP1 kinase inhibitor, abolishes Smac mimetic- and TNFα-induced cell death in FADD- or caspase-8-deficient. Thus, Smac mimetic enhances TNFα-induced cell death in leukemia cells via two distinct pathways in a context-dependent manner: it primes apoptosis-resistant cells lacking FADD or caspase-8 to TNFα-induced, RIP1-dependent and caspase-independent necroptosis, whereas it sensitizes apoptosis-proficient cells to TNFα-mediated, caspase-dependent apoptosis. These findings have important implications for the therapeutic exploitation of necroptosis as an alternative cell death program to overcome apoptosis resistance.

  5. Circadian variation in serum free and total insulin-like growth factor (IGF)-I and IGF-II in untreated and treated acromegaly and growth hormone deficiency

    DEFF Research Database (Denmark)

    Skjaerbaek, Christian; Frystyk, Jan; Kaal, Andreas

    2000-01-01

    to the nocturnal increase in IGF binding protein-1. In this study we have investigated the circadian variation in circulating free IGF-I and IGF-II in patients with acromegaly and patients with adult onset growth hormone deficiency. PATIENTS: Seven acromegalic patients were studied with and without treatment...... no significant circadian variations in free IGF-I or free IGF-II in either of the two occasions. In contrast, there was a significant circadian variation of total IGF-I after adjustment for changes in plasma volume in both treated and untreated acromegaly and GH deficiency in all cases with a peak between 0300 h...

  6. Circadian variation in serum free and total insulin-like growth factor (IGF)-I and IGF-II in untreated and treated acromegaly and growth hormone deficiency

    DEFF Research Database (Denmark)

    Skjaerbaek, Christian; Frystyk, Jan; Kaal, Andreas;

    2000-01-01

    to the nocturnal increase in IGF binding protein-1. In this study we have investigated the circadian variation in circulating free IGF-I and IGF-II in patients with acromegaly and patients with adult onset growth hormone deficiency. PATIENTS: Seven acromegalic patients were studied with and without treatment...... no significant circadian variations in free IGF-I or free IGF-II in either of the two occasions. In contrast, there was a significant circadian variation of total IGF-I after adjustment for changes in plasma volume in both treated and untreated acromegaly and GH deficiency in all cases with a peak between 0300 h...

  7. Fibromodulin-deficiency alters temporospatial expression patterns of transforming growth factor-β ligands and receptors during adult mouse skin wound healing.

    Directory of Open Access Journals (Sweden)

    Zhong Zheng

    Full Text Available Fibromodulin (FMOD is a small leucine-rich proteoglycan required for scarless fetal cutaneous wound repair. Interestingly, increased FMOD levels have been correlated with decreased transforming growth factor (TGF-β1 expression in multiple fetal and adult rodent models. Our previous studies demonstrated that FMOD-deficiency in adult animals results in delayed wound closure and increased scar size accompanied by loose package collagen fiber networks with increased fibril diameter. In addition, we found that FMOD modulates in vitro expression and activities of TGF-β ligands in an isoform-specific manner. In this study, temporospatial expression profiles of TGF-β ligands and receptors in FMOD-null and wild-type (WT mice were compared by immunohistochemical staining and quantitative reverse transcriptase-polymerase chain reaction using a full-thickness, primary intention wound closure model. During the inflammatory stage, elevated inflammatory infiltration accompanied by increased type I TGF-β receptor levels in individual inflammatory cells was observed in FMOD-null wounds. This increased inflammation was correlated with accelerated epithelial migration during the proliferative stage. On the other hand, significantly more robust expression of TGF-β3 and TGF-β receptors in FMOD-null wounds during the proliferative stage was associated with delayed dermal cell migration and proliferation, which led to postponed granulation tissue formation and wound closure and increased scar size. Compared with WT controls, expression of TGF-β ligands and receptors by FMOD-null dermal cells was markedly reduced during the remodeling stage, which may have contributed to the declined collagen synthesis capability and unordinary collagen architecture. Taken together, this study demonstrates that a single missing gene, FMOD, leads to conspicuous alternations in TGF-β ligand and receptor expression at all stages of wound repair in various cell types. Therefore

  8. Risk factors for prevalence of folic acid deficiency in Chinese lactating women in 2013%2013年中国乳母叶酸缺乏状况及其影响因素

    Institute of Scientific and Technical Information of China (English)

    毕烨; 段一凡; 王杰; 于冬梅; 杨晓光; 荫士安; 杨振宇

    2016-01-01

    目的:了解2013年中国乳母叶酸缺乏状况,并分析影响叶酸缺乏的因素。方法数据来源于2013年中国居民营养与健康状况监测,采用多阶段分层整群抽样方法,调查对象为中国内地除西藏自治区以外的30个省(直辖市、自治区)的55个县(市/区)产后0~24个月的乳母,总样本量为10331名。采用问卷调查收集乳母基本情况,通过膳食频率调查收集受试者近1个月的膳食信息。按照单纯随机抽样方法抽取20%的乳母,采用电化学发光法测定血清叶酸水平,将血清叶酸水平 acid deficiency was 0.3% (1/388), 1.3% (7/550), 6.0% (38/639) and 3.2% (10/317) in larger, medium or small cities, general rural counties and poor rural counties, respectively. The prevalence of folic acid deficiency was 6.3% (17/269) and 2.4% (39/1 623) for minority and Han ethnic groups, respectively, and was 6.3% (52/823) and 0.4% (4/1 071) for subjects in northern and southern areas of China, respectively. Binary unconditionally logistic regression was used to analyze the factors associated with folic acid deficiency in the lactating women. The results showed that, compared with those living in the south and those in the Han ethnic group, those living in the north and belonging to the minority ethnic group had a greater risk of folic acid deficiency, with (OR=3.63, 95%CI: 1.64-8.05) and (OR=5.42, 95%CI: 1.75-16.74), respectively. With each month of age during childhood, the prevalence of folic acid deficiency decreased by 10%(OR=0.90, 95%CI:0.85-0.96). Compared with low income subjects, those with middle incomes had OR (95%CI) of 0.25 (0.10-0.60) for folic acid deficiency. A lesser intake of cereal was associated with a greater risk of folic acid deficiency in lactating women (OR=3.04, 95%CI:1.18-7.79). A lesser intake of tubers was a protective factor for folic acid deficiency in lactating women (OR=0.29, 95%CI:0.12-0.70). Conclusion The folic acid status of

  9. Vitamin B12 status in older adults living in Ontario long-term care homes: prevalence and incidence of deficiency with supplementation as a protective factor.

    Science.gov (United States)

    Pfisterer, Kaylen J; Sharratt, Mike T; Heckman, George G; Keller, Heather H

    2016-02-01

    Vitamin B12 (B12) deficiency, although treatable, impacts up to 43% of community-living older adults; long-term care (LTC) residents may be at greater risk. Recommendations for screening require further evidence on prevalence and incidence in LTC. Small, ungeneralizable samples provide a limited perspective on these issues. The purposes of this study were to report prevalence of B12 deficiency at admission to LTC, incidence 1 year post-admission, and identify subgroups with differential risk. This multi-site (8), retrospective prevalence study used random proportionate sampling of resident charts (n = 412). Data at admission extracted included demographics, B12 status, B12 supplementation, medications, diagnoses, functional independence, cognitive performance, and nutrition. Prevalence at admission of B12 deficiency (B12 (>300 pmol/L). One year post-admission incidence was 4%. Better B12 status was significantly associated with supplementation use prior to LTC admission. Other characteristics were not associated with status. This work provides a better estimate of B12 deficiency prevalence than previously available for LTC, upon which to base protocols and policy. Prospective studies are needed to establish treatment efficacy and effect on health related outcomes.

  10. Signatures of the non-Maxwellian $\\kappa$-distributions in optically thin line spectra \\subtitle{I. Theory and synthetic Fe IX--XIII spectra}

    CERN Document Server

    Dudík, J; Mason, H E; Dzifčáková, E

    2014-01-01

    We investigate the possibility of diagnosing the degree of departure from the Maxwellian distribution using single-ion spectra originating in astrophysical plasmas in collisional ionization equilibrium. New atomic data for excitation of Fe IX-XIII are integrated under the assumption of a kappa-distribution of electron energies. Diagnostic methods using lines of a single ion formed at any wavelength are explored. Such methods minimize uncertainties from the ionization and recombination rates, as well as the possible presence of non-equilibrium ionization. Approximations to the collision strengths are also investigated. The calculated intensities of most of the Fe IX-XIII EUV lines show consistent behaviour with kappa at constant temperature. Intensities of these lines decrease with kappa, with the vast majority of ratios of strong lines showing little or no sensitivity to kappa. Several of the line ratios, especially involving temperature-sensitive lines, show a sensitivity to kappa that is of the order of sev...

  11. Palavra de púlpito e erudição no século XIII: A Legenda aurea de Jacopo de Varazze

    Directory of Open Access Journals (Sweden)

    Néri de Almeida Souza

    2002-01-01

    Full Text Available A partir da consideração do mais importante exemplar da hagiografia medieval, a Legenda aurea do dominicano Jacopo de Varazze, o texto pretende discorrer sobre os diversos níveis de erudição dos clérigos cristãos no século XIII e sua importância para a consideração das fontes documentais do período.This article intends to deal with the most important text of medieval hagiography, the Jacopo de Varazze's Legenda Aurea. Our main goal is to demonstrate the christian clergymen's erudition in the XIII century and their importance as historical sources of the period.

  12. Carnitine Deficiency and Pregnancy

    OpenAIRE

    Anouk de Bruyn; Yves Jacquemyn; Kristof Kinget; François Eyskens

    2015-01-01

    We present two cases of carnitine deficiency in pregnancy. In our first case, systematic screening revealed L-carnitine deficiency in the first born of an asymptomatic mother. In the course of her second pregnancy, maternal carnitine levels showed a deficiency as well. In a second case, a mother known with carnitine deficiency under supplementation was followed throughout her pregnancy. Both pregnancies had an uneventful outcome. Because carnitine deficiency can have serious complications, su...

  13. La moneda castellana en los manuales de mercaderías y tratados de aritmética italianos bajomedievales (siglos XIII-XV)

    OpenAIRE

    2007-01-01

    El presente artículo analiza el conocimiento sobre la moneda y el sistema monetario vigentes en Castilla en la Baja Edad Media en los manuales de mercadería y los tratados de aritmética italianos de los siglos XIII al XV. The present paper analyzes the knowledge of money and money system in Castile in the Late Middle Ages in the Italian pratiche di mercatura and treaties of Arithmetics from the XIIIth to XVth Centuries.

  14. Functionalization based on the substitutional flexibility: strong middle IR nonlinear optical selenides AX(II)(4)X(III)(5)Se12.

    Science.gov (United States)

    Lin, Hua; Chen, Ling; Zhou, Liu-Jiang; Wu, Li-Ming

    2013-08-28

    Seven nonlinear optical (NLO) active selenides in the middle IR region, AX(II)4X(III)5Se12 (A = K(+)-Cs(+); X(II) = Mn(2+), Cd(2+); X(III) = Ga(3+), In(3+)) adopting the KCd4Ga5S12-type structure, have been synthesized by high-temperature solid-state reaction of an elemental mixture with ACl flux. Their three-dimensional network structures are stacked by M9Se24-layers of vertex sharing MSe4 tetrahedra, of which each center is jointly occupied by X(II) and X(III) atoms. Studies suggest that such tetrahedral building units can be regarded as the "multi-functional sites", on which the Cd(2+)/Ga(3+) pair gives rise to the coexistence of NLO and thermochromic properties, and the Mn(2+)/In(3+) pair leads to the coexistence of NLO and magnetic properties. The density functional theory (DFT) studies and the cutoff-energy-dependent NLO coefficient analyses reveal that such "multi-functional sites" contribute to the origin of the second harmonic generation (SHG) that is ascribed to the electronic transitions from the Se-4p states to the ns, np states of X(II) and X(III) atoms. Remarkably, title compounds show very strong SHG at an incident wavelength of 2.05 μm, roughly 16-40 times that of commercial AgGaS2; among them, ACd4In5Se12 (A = Rb, Cs) represents the strongest SHG among chalcogenides to date.

  15. La moneda castellana en los manuales de mercaderías y tratados de aritmética italianos bajomedievales (siglos XIII-XV)

    OpenAIRE

    González Arévalo, Raúl

    2007-01-01

    El presente artículo analiza el conocimiento sobre la moneda y el sistema monetario vigentes en Castilla en la Baja Edad Media en los manuales de mercadería y los tratados de aritmética italianos de los siglos XIII al XV. The present paper analyzes the knowledge of money and money system in Castile in the Late Middle Ages in the Italian pratiche di mercatura and treaties of Arithmetics from the XIIIth to XVth Centuries.

  16. Differential regulation of hepatic transcription factors in the Wistar rat offspring born to dams fed folic acid, vitamin B12 deficient diets and supplemented with omega-3 fatty acids.

    Directory of Open Access Journals (Sweden)

    Akshaya Meher

    Full Text Available Nutritional status of the mother is known to influence various metabolic adaptations required for optimal fetal development. These may be mediated by transcription factors like peroxisome proliferator activated receptors (PPARs, which are activated by long chain polyunsaturated fatty acids. The objective of the current study was to examine the expression of different hepatic transcription factors and the levels of global methylation in the liver of the offspring born to dams fed micronutrient deficient (folic acid and vitamin B12 diets and supplemented with omega-3 fatty acids. Female rats were divided into five groups (n = 8/group as follows; control, folic acid deficient (FD, vitamin B12 deficient (BD and omega-3 fatty acid supplemented groups (FDO and BDO. Diets were given starting from pre-conception and continued throughout pregnancy and lactation. Pups were dissected at the end of lactation. Liver tissues were removed; snap frozen and stored at -80°C. Maternal micronutrients deficiency resulted in lower (p<0.05 levels of pup liver docosahexaenoic acid (DHA and arachidonic acid (ARA as compared to the control group. Pup liver PPARα and PPARγ expression was lower (p<0.05 in the BD group although there were no differences in the expression of SREBP-1c, LXRα and RXRα expression. Omega-3 fatty acids supplementation to this group normalized (p<0.05 levels of both PPARα and PPARγ but reduced (p<0.05 SREBP-1c, LXRα and RXRα expression. There was no change in any of the transcription factors in the pup liver in the FD group. Omega-3 fatty acids supplementation to this group reduced (p<0.05 PPARα, SREBP-1c and RXRα expression. Pup liver global methylation levels were higher (p<0.01 in both the micronutrients deficient groups and could be normalized (p<0.05 by omega-3 fatty acid supplementation. Our novel findings suggest a role for omega-3 fatty acids in the one carbon cycle in influencing the hepatic expression of transcription factors

  17. PREFACE: XIII International Conference on Calorimetry in High Energy Physics (CALOR 2008)

    Science.gov (United States)

    Livan, Michele

    2009-07-01

    The XIII International Conference on Calorimetry in High Energy Physics was held in Pavia, Italy, 26-30 May 2008, picking up the baton from the 2006 Conference in Chicago. The Conference took place in the unique environment of the Theresian Room of the University Library. The attendees were surrounded by over 40 000 books of general interest and culture, and had the opportunity to see precious volumes written by such people as Galileo, Volta and Faraday. The Workshop brought together more than 120 participants, including senior scientists as well as young physicists, confirming the central and ever-growing role of calorimeters in modern particle physics. The development of these detectors, as stressed by Professor Klaus Pretzl in his lectio magistralis, has made it possible to explore new frontiers in physics, and the present scenario is no exception to this rule. With the LHC experiments almost completely installed and ready to take data, the Conference was an ideal chance to review the status of the different projects, whose development has been followed and discussed throughout the entire Calor series, and to show that they are capable of meeting the design specifications. Other highlights were the performance and physics results of calorimeters installed in currently operating experiments. In the session on astrophysics and neutrinos, the contributions confirmed the key role of calorimeters in this sector and demonstrated their growing application even beyond the field of accelerator physics. Considerable time was devoted to the state-of-the-art techniques in the design and operation of the detectors, while the session on simulation addressed the importance of a thorough understanding of the shower development to meet the demanding requirements of present experiments. Finally, on the R&D side, the particle flow and dual read-out concepts confronted the challenges issued by the next generation of experiments. This complex material was reviewed in 83

  18. PREFACE: XIII International Seminar on Physics and Chemistry of Solids (ISPCS)

    Science.gov (United States)

    Berdowski, Janusz

    2007-06-01

    This volume of Journal of Physics: Conference Series contains some of the papers which were presented at the XIII International Seminar on Physics and Chemistry of Solids (ISPCS) in June 2007, in Ustroń, Poland. As the materials from ISPCS are presented in this Journal for the first time it is a good opportunity to give a brief outline of the Seminar's roots, history and goals. The initiator of the Seminars, conceived as annual meetings of the physicists and chemists from Ukraine and Poland, was the late Professor of the Ivan Franko National University in Lviv, Wlodymyr Sawicki. As Professor Sawicki had also lectured for the students of Jan Dlugosz University in Czȩstochowa he had seen both these universities as future organizers of the conference. Coincidentally rectors of Lviv and Czȩstochowa universities, Professor Ivan Vakarchuk and Professor Józef Światek were physicists so this proposition was received very warmly and got strong support from the officials of the universities. From the early beginnings the Seminar also had wide organizational help from the Research and Development Enterprise 'Carat' from Lviv and especially from its president Dr Mykola Vakiv. The Seminars started in 1996—the first meeting took place in Zakopane (Poland) in May 1996 and the second one in September of the same year in Schack (Ukraine). From 1997, ISPCS Seminars have gathered together a group of chemists and physicists interested in condensed matter physics and chemistry, in even years in Ukraine, in odd years in Poland. This circle is growing: at the first Seminar in Zakopane thirty scientists took part, mainly from Lviv and from Czȩstochowa, ISPCS13 gave us the opportunity to meet over eighty people from several universities and research institutions, including delegates from countries other than Poland and Ukraine, with over seventy presentations. The organizers plan that ISPCS conferences should achieve the following two objectives: help in building closer

  19. Real-time PCR demonstrates Ancylostoma duodenale is a key factor in the etiology of severe anemia and iron deficiency in Malawian pre-school children.

    Directory of Open Access Journals (Sweden)

    Femkje A M Jonker

    Full Text Available BACKGROUND: Hookworm infections are an important cause of (severe anemia and iron deficiency in children in the tropics. Type of hookworm species (Ancylostoma duodenale or Necator americanus and infection load are considered associated with disease burden, although these parameters are rarely assessed due to limitations of currently used diagnostic methods. Using multiplex real-time PCR, we evaluated hookworm species-specific prevalence, infection load and their contribution towards severe anemia and iron deficiency in pre-school children in Malawi. METHODOLOGY AND FINDINGS: A. duodenale and N. americanus DNA loads were determined in 830 fecal samples of pre-school children participating in a case control study investigating severe anemia. Using multiplex real-time PCR, hookworm infections were found in 34.1% of the severely anemic cases and in 27.0% of the non-severely anemic controls (p<0.05 whereas a 5.6% hookworm prevalence was detected by microscopy. Prevalence of A. duodenale and N. americanus was 26.1% and 4.9% respectively. Moderate and high load A. duodenale infections were positively associated with severe anemia (adjusted odds ratio: 2.49 (95%CI 1.16-5.33 and 9.04 (95%CI 2.52-32.47 respectively. Iron deficiency (assessed through bone marrow examination was positively associated with intensity of A. duodenale infection (adjusted odds ratio: 3.63 (95%CI 1.18-11.20; 16.98 (95%CI 3.88-74.35 and 44.91 (95%CI 5.23-385.77 for low, moderate and high load respectively. CONCLUSIONS/SIGNIFICANCE: This is the first report assessing the association of hookworm load and species differentiation with severe anemia and bone marrow iron deficiency. By revealing a much higher than expected prevalence of A. duodenale and its significant and load-dependent association with severe anemia and iron deficiency in pre-school children in Malawi, we demonstrated the need for quantitative and species-specific screening of hookworm infections. Multiplex real

  20. Correlation factors of nutritional deficiencies in critically ill children%危重患儿营养支持不足及相关因素分析

    Institute of Scientific and Technical Information of China (English)

    沈丽媛; 赵醴; 洪莉; 冯一; 潘莉雅; 徐建蓉

    2013-01-01

    Objective To assess the nutritional status of critically ill children and the nutritional intake during their stay in pediatric intensive care unit (PICU),and analyze the correlation factors of nutritional deftciencies.Methods One hundred and twenty-seven patients who met the PICU admission standards with a PICU stay of >72 h were recruited from June to October 2012 in Shanghai Children's Medical Center.Anthropometric measurements and all nutrition-related indicators of those patients were recorded from day 1 to day 10.Results 59 of the 127 patients (46.5%) showed malnutrition at admission,in which 49.2% had severe malnutrition.65 of the 127 patients (51.2%) showed malnutrition at discharge,in which 63.1% were severely malnourished.Median estimated energy requirements (EER) by American Society for Parenteral and Enteral Nutrition was 376.7 kJ/ (kg · d) [interquartile range,IQR:314.0~376.7 kJ/ (kg · d)],prescribed calories were 237.8 kJ/ (kg· d) [IQR:159.5 ~291.8 kJ/ (ks· d)],and delivered calories were 220.2 kJ/ (kg· d)[IQR:132.3 ~ 279.2 k J/ (kg · d)],showing significant difference (P =0.000).The delivered energy was <90% of EER in 80.7% of the 1021 recorded days and the prescribed energy was <90% of EER in 74.3%of the 1021 recorded days.The cumulative calory deficiency from day 1 to day 10 in PICU was (933.5 ±745.5) kJ/ (kg · person),and the cumulative protein deficiency was (4.0 ±5.0) g/ (kg · person).83 patients (65.4%) experienced at least one feeding interruption.Altogether 170 times of feeding interruption were recorded,of which 117 (68.8%) could be explained by examination procedures.Conclusions There is a high prevalence of malnutrition in critically ill children at admission into PICU,and their nutritional status deteriorates during hospital stay.Discrepancies between required and delivered energy were mainly attributed to under-prescription,while discrepancies between prescribed and delivered energy were mainly

  1. THE ROLE OF PSYCHOPHYSIOLOGIC AND ANTHROPOMETRIC DESCRIPTIONS IN THE BEGINNING OF PSYCHOSOMATIC PATHOLOGY: IS THE DEFICIENCY OF BODY WEIGHT A RISK FACTOR?

    Directory of Open Access Journals (Sweden)

    T.I. Mareeva

    2007-09-01

    Full Text Available In order to find out psychophysiologic preconditions for the beginning of psychosomatic pathology, 264 clinically healthy persons of both sexes were examined (the average age was 22,16 + 2,42. All of them were divided into 2 groups according to their body weight index: the first group had normal body weight index, the second had deficiency of body weight. With the help of psychodiagnostic methods (Reduced Multiple Questionnaire for the examining of the Personality, Psychogeometrical test, Spielberger's test, Subbotin's test, Ekkles's test it was found that persons with deficiency of body weight had had conflicting personality features, which form prepsychosomatic radical of personality. It underlies the development of psychosomatics diseases and deviant behavior.

  2. Zinc-Dependent Lysosomal Enlargement in TRPML1-Deficient Cells Involves MTF-1 Transcription Factor and ZnT4 (Slc30a4) Transporter

    OpenAIRE

    2013-01-01

    Zn is critical for a multitude of cellular processes, including gene expression, secretion and enzymatic activities. Cellular Zn is controlled by Zn-chelating proteins and by Zn transporters. The recent identification of Zn permeability of the lysosomal ion channel TRPML1, and the evidence of abnormal Zn levels in cells deficient in TRPML1, suggested a role for TRPML1 in Zn transport. Here we provide new evidence for such a role and identify additional cellular components responsible for it. ...

  3. [Phosphate metabolism and iron deficiency].

    Science.gov (United States)

    Yokoyama, Keitaro

    2016-02-01

    Autosomal dominant hypophosphatemic rickets(ADHR)is caused by gain-of-function mutations in FGF23 that prevent its proteolytic cleavage. Fibroblast growth factor 23(FGF23)is a hormone that inhibits renal phosphate reabsorption and 1,25-dihydroxyvitamin D biosynthesis. Low iron status plays a role in the pathophysiology of ADHR. Iron deficiency is an environmental trigger that stimulates FGF23 expression and hypophosphatemia in ADHR. It was reported that FGF23 elevation in patients with CKD, who are often iron deficient. In patients with nondialysis-dependent CKD, treatment with ferric citrate hydrate resulted in significant reductions in serum phosphate and FGF23.

  4. Efeito do fator XIII da coagulação na cicatrização da pele de ratos em uso de corticosteróide

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    Aldo da Cunha Medeiros

    Full Text Available OBJETIVOS: Observar o efeito do fator XIII da coagulação (Fibrogamin® na cicatrização de feridas incisas da pele de ratos tratados com corticosteróide. Foi feita a avaliação quanto ao aspecto histopatológico dos tecidos em cicatrização e sua resistência à tensão. MÉTODO: Foram utilizados 40 ratos Wistar, divididos em quatro grupos. No grupo A (n=10, foi administrado corticosteróide. No grupo B (n=10 foi usado corticosteróide e fator XIII. No grupo C (n=10 foi injetado fator XIII e no grupo D (n=10 foi administrado placebo (controle. A resistência à tensão foi medida através de tensiômetro computadorizado e as alterações histopatológicas quantificadas por análise digital. RESULTADOS: Ocorreu uma significativa diminuição da resistência da ferida de pele no grupo A (523,6gf, quando comparado com o controle (1480,4gf. No grupo B (868,8gf notou-se significativa diferença em relação ao grupo A (p<0,0001. O grupo C não mostrou diferença (p=0,067 em relação ao grupo controle (D, entretanto foram observadas diferenças significativas quando comparados os grupos A e C; A e D (p<0,0001. A análise da densidade do colágeno e de células inflamatórias revelou as mesmas diferenças observadas na resistência à tensão. CONCLUSÕES: Foi observado que a ação do corticosteróide dificultou a cicatrização da pele de ratos e diminuiu a resistência à tensão, ação revertida pelo uso do fator XIII . A utilização do fator XIII sem uso de corticosteróide não demonstrou ação de melhora nos resultados da cicatrização em relação ao controle.

  5. La expansión del posesivo pospuesto a la luz de documentación navarra (y aragonesa del siglo XIII

    Directory of Open Access Journals (Sweden)

    Ángeles Romero Cambrón

    2016-06-01

    Full Text Available En los primitivos documentos romances el posesivo sólo aparece antepuesto al sustantivo (“(el nuestro padre”. La posposición (“padre nuestro” se empieza a registrar en el siglo XIII en aragonés y en dialecto navarro. Considerando principalmente la documentación de esta segunda variedad, el presente trabajo tiene como objetivo determinar el modo en que el posesivo adquirió la capacidad de aparecer pospuesto. Este cambio se vio impulsado por factores de naturaleza estructural. Uno de ellos es la existencia de la construcción posesiva con de y pronombre personal (“por raçón de mí”; otro, la concurrencia del posesivo en coordinación, bien con el sintagma preposicional (“nin subdito vuestro nin de la eglesia”, bien con otro posesivo (“l’amor nuestra e vuestra”. El posesivo se pospondría adoptando la posición propia del sintagma preposicional. Asimismo, habría influido en la imposición del cambio la tendencia a aligerar la posición prenominal cuando concurrían en ella más de un adyacente. In the first Romance texts, possessives only occur in prenominal position (e.g. (el nuestro padre. The earliest records of postposition (e.g. padre nuestro date back to the 13th century in Aragonese and in Navarrese dialect. This paper reports largely on documentation involving Navarrese in order to determine in what way the postpositive placement of the possessive became normal. The influence of three grammar structures has been found to trigger possessive postposition. The first structure is a possessive construction consisting of the preposition de followed by a personal pronoun (e.g. campo de nos. The second structure includes a possessive in coordination either with a prepositional phrase (nin subdito vuestro nin de la eglesia or with another possessive (l’amor nuestra e vuestra. In this case, the possessive occurs after the noun in the natural place of a prepositional phrase. Finally, possessive postposition became a

  6. 影响橡胶树容器苗缺铁黄化的因素及矫正技术%Factors Influencing Iron Deficiency Chlorosis of Container Rubber Seedlings and Correction Technique

    Institute of Scientific and Technical Information of China (English)

    张能; 黄志; 潘媛; 李彭怡

    2011-01-01

    对影响橡胶树容器苗缺铁黄化的几个因素进行了研究,并使用不同补铁方法对缺铁黄化进行矫正效果比较。结果表明:缺铁黄化全年均可发生,以高温季节发生严重;培养土配方塘泥/园土=1/4(V/V)能减轻黄化;砧穗组合东方93-114/云研77-4对缺铁最敏感;使用0.2%螯合铁灌根矫正效果较好。%By means of studying the main factors causing iron deficiency chlorosis and comparing the remedy effects of different ion preparations on rubber tree container seedlings.The results showed that iron deficiency chlorosis can occur in the whole year and reach peaks during high temperature season.The effects of the various iron preparations were different.The nutritive soil recipe for cultivation of rubber tree container seedlings was the components of pond sludge/garden soil=1/4 can reduce the etiolation;Rootstock-scion combinations of DongFang 93-114/YunYan77-4 was most sensitive to iron deficiency;The chelating iron for irrigating roots was much better than others.

  7. Deficiência de ferro, prevalência de anemia e fatores associados em crianças de creches públicas do oeste do Paraná, Brasil Iron deficiency and prevalence of anemia and associated factors in children attending public daycare centers in western Paraná, Brazil

    Directory of Open Access Journals (Sweden)

    Valdete Carreira Rodrigues

    2011-06-01

    Full Text Available OBJETIVO: Avaliar o estado nutricional de ferro, a prevalência de anemia e fatores associados, em crianças de 6 a 24 meses frequentadoras de creche pública em Cascavel, Região Oeste do Paraná, Brasil. MÉTODOS: O estudo transversal foi realizado com amostra aleatória de 256 crianças. A coleta de dados (questionário, medidas antropométricas e amostras de sangue ocorreu de julho a setembro de 2007. A deficiência de ferro foi avaliada em termos de transferrina, hemoglobina, volume corpuscular médio, ferro sérico e eosinófilos. Na análise estatística dos dados foram obtidas as odds ratio bruta e ajustada (regressão logística, bem como os respectivos níveis de significância (p-valor. Para identificar diferenças significativas entre as medidas quantitativas, adotou-se a Análise de Variância e o teste de comparação múltipla de Tukey. RESULTADOS: A prevalência da anemia foi de 29,7%, sendo que 77,3% das amostras apresentaram baixa concentração de ferro. A antropometria não apontou deficiência de macronutrientes, porém mostrou obesidade acima dos índices médios. Os fatores associados à anemia e à deficiência de ferro foram: doenças frequentes na família (OR=10,02, condições de moradia (OR=5,05, tempo de creche (OR=3,05, número de moradores na residência (OR=2,83 e falta de saneamento (OR=2,20. CONCLUSÃO: A prevalência de anemia e a elevada deficiência de ferro detectada evidenciam um grave problema de saúde pública entre os pré-escolares do município de Cascavel, Paraná. Apesar da amplitude do problema, a anemia não está sendo reconhecida, prevenida e tratada adequadamente. Neste estudo são sugeridas algumas possíveis intervenções.OBJECTIVE: This study assessed the iron levels and prevalence of anemia and associated factors in children aged 6 to 24 months attending public daycare centers in Cascavel, Western Paraná, Brazil. METHODS: This cross-sectional study included 256 randomly sampled children

  8. Alterations of epigenetic signatures in hepatocyte nuclear factordeficient mouse liver determined by improved ChIP-qPCR and (h)MeDIP-qPCR assays.

    Science.gov (United States)

    Zhang, Qinghao; Lei, Xiaohong; Lu, Hong

    2014-01-01

    Hepatocyte nuclear factor 4α (HNF4α) is a liver-enriched transcription factor essential for liver development and function. In hepatocytes, HNF4α regulates a large number of genes important for nutrient/xenobiotic metabolism and cell differentiation and proliferation. Currently, little is known about the epigenetic mechanism of gene regulation by HNF4α. In this study, the global and specific alterations at the selected gene loci of representative histone modifications and DNA methylations were investigated in Hnf4a-deficient female mouse livers using the improved MeDIP-, hMeDIP- and ChIP-qPCR assay. Hnf4a deficiency significantly increased hepatic total IPed DNA fragments for histone H3 lysine-4 dimethylation (H3K4me2), H3K4me3, H3K9me2, H3K27me3 and H3K4 acetylation, but not for H3K9me3, 5-methylcytosine,or 5-hydroxymethylcytosine. At specific gene loci, the relative enrichments of histone and DNA modifications were changed to different degree in Hnf4a-deficient mouse liver. Among the epigenetic signatures investigated, changes in H3K4me3 correlated the best with mRNA expression. Additionally, Hnf4a-deficient livers had increased mRNA expression of histone H1.2 and H3.3 as well as epigenetic modifiers Dnmt1, Tet3, Setd7, Kmt2c, Ehmt2, and Ezh2. In conclusion, the present study provides convenient improved (h)MeDIP- and ChIP-qPCR assays for epigenetic study. Hnf4a deficiency in young-adult mouse liver markedly alters histone methylation and acetylation, with fewer effects on DNA methylation and 5-hydroxymethylation. The underlying mechanism may be the induction of epigenetic enzymes responsible for the addition/removal of the epigenetic signatures, and/or the loss of HNF4α per se as a key coordinator for epigenetic modifiers.

  9. Parent Stars of Extrasolar Planets - XIII. Additional Evidence for Li Abundance Anomalies

    CERN Document Server

    Gonzalez, Guillermo

    2014-01-01

    We report the results of our analysis of new high resolution spectra of 37 late-F to early-G dwarf stars for the purpose of deriving their Li abundances. Most of the stars were selected from the large Valenti and Fischer compilation and had unknown Li abundances prior to the present study. When the new data are combined with data from our previous studies on this topic and analyzed in a similar way, we find, again, that stars with planets near the solar temperature are deficient in Li relative to a comparison set of stars. A similar result is obtained when we combine our data with a large database of stellar Li abundances from the literature.

  10. Psychomotor development in children with iron deficiency and iron-deficiency anemia.

    Science.gov (United States)

    Pala, Emin; Erguven, Muferet; Guven, Sirin; Erdogan, Makbule; Balta, Tulin

    2010-09-01

    Iron deficiency and iron-deficiency anemia are the most common nutritional deficiencies in children, especially in developing countries. Iron-deficiency anemia in infancy is associated with impaired neurodevelopment. Studies have shown an association between iron deficiency without anemia and adverse effects on psychomotor development. To determine the effects of iron deficiency and iron-deficiency anemia on psychomotor development in childhood. . We evaluated psychomotor development in healthy children with iron deficiency and iron-deficiency anemia with the use of the Denver II Developmental Screening Test (DDST-II). If the child score was more than 90th percentile compared to children in the same age group, the test was scored as "delay" it was scored as a "caution" if the child score was between the 75th and 90th percentiles. The test result was interpreted as "normal," if there was no delay and only one "caution" for any item. If the child had one or more "delays" or more than two "cautions," the result was classified as "abnormal." DDST-II scores were abnormal in 67.3% of subjects with iron-deficiency anemia, 21.6% of those with iron deficiency, and 15.0% of control subjects. The difference from the control group in the percentage of abnormal scores was significant for subjects with iron-deficiency anemia (p iron deficiency (p = 0.203); p > .05. (p-value, post-hoc comparison of 2 groups.) Iron-deficiency anemia impaired psychomotor development during childhood. However, the evidence on the adverse effects of iron deficiency remains controversial. The Denver II Developmental Screening Test is a valuable test to detect early developmental delays, especially in infants with risk factors.

  11. Normal growth spurt and final height despite low levels of all forms of circulating insulin-like growth factor-I in a patient with acid-labile subunit deficiency

    DEFF Research Database (Denmark)

    Domené, Horacio M; Martínez, Alicia S; Frystyk, Jan

    2007-01-01

    deficiency and determined both spontaneous and growth hormone (GH)-stimulated changes in the IGF system, including measurements of total, free and bioactive IGF-I, total IGF-II and insulin-like growth factor binding protein (IGFBP)-1, IGFBP-2 and IGFBP-3. RESULTS: The patient had a delayed growth...... as after puberty, basal levels of total, free and bioactive IGF-I were low, as were total IGF-II, IGFBP-1, IGFBP-2 and IGFBP-3. GH treatment for 6 months normalized free IGF-I and increased bioactive IGF-I, but had no effect on growth velocity. CONCLUSIONS: This case story shows that in the presence...

  12. Familial lipoprotein lipase deficiency

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000408.htm Familial lipoprotein lipase deficiency To use the sharing features on this page, please enable JavaScript. Familial lipoprotein lipase deficiency is a group of rare genetic ...

  13. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... This Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video— ... treatment. For more information about living with and managing iron-deficiency anemia, go to the Health Topics ...

  14. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... or an inability to absorb enough iron from food. Overview Iron-deficiency anemia is a common type ... of the condition. Treatments may include dietary changes, medicines, and surgery. Severe iron-deficiency anemia may require ...

  15. Folate-deficiency anemia

    Science.gov (United States)

    ... medlineplus.gov/ency/article/000551.htm Folate-deficiency anemia To use the sharing features on this page, please enable JavaScript. Folate-deficiency anemia is a decrease in red blood cells (anemia) ...

  16. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... severity of the condition. Treatments may include dietary changes, medicines, and surgery. Severe iron-deficiency anemia may require treatment in a hospital, blood ... With and Managing Iron-Deficiency Anemia 05/18/2011 This video— ...

  17. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... or an inability to absorb enough iron from food. Overview Iron-deficiency anemia is a common type ... condition. Treatments may include dietary changes, medicines, and surgery. Severe iron-deficiency anemia may require treatment in ...

  18. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Deficiency Anemia What Is... CAUSES WHO IS AT RISK SIGNS & SYMPTOMS DIAGNOSIS TREATMENTS PREVENTION LIVING WITH CLINICAL ... and women are the two groups at highest risk for iron-deficiency anemia. Outlook Doctors usually can ...

  19. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Deficiency Anemia Explore Iron-Deficiency Anemia What Is... CAUSES WHO IS AT RISK SIGNS & SYMPTOMS DIAGNOSIS TREATMENTS ... Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG CONTACT US National Institutes of Health ...

  20. A monarquia como império de si: o caso da realeza de D. Dinis (Séc. XIII

    Directory of Open Access Journals (Sweden)

    Carlos Eduardo Zlatic

    2013-03-01

    Full Text Available O reinado de D. Dinis (1261 – 1325, rei desde 1279 é entendido pela historiografia portuguesa como marco na construção do Estado Moderno em Portugal, devido ao processo de centralização política efetivada por esse rei. Considerando essa interpretação, o presente artigo pretende apontar as influências exercidas pelo horizonte político do século XIII no Ocidente Medieval - marcado pela afirmação das monarquias feudais sobre os poderes da Igreja e do Império - e no reino de Portugal – aonde a Reconquista chegara ao fim – sobre as ações régias. Com este objetivo, o texto discorrerá sobre como o ideário político desse contexto legitimou a autoridade e a função do rei como fonte primeira de justiça no interior de sues domínios e legitimou a busca pelo fortalecimento da Coroa Portuguesa frente às forças externas e internas ao âmbito próprio de sua jurisdição.

  1. La evolución de la espada en la sociedad catalana de los siglos XI al XIII

    Directory of Open Access Journals (Sweden)

    Cirlot, Maria Victoria

    1978-12-01

    Full Text Available Not available.

    EL estudio del armamento tiene su origen en las mismas exigencias de la historia social. Cada vez más, se hace inevitable la minuciosa reconstrucción de aquellos elementos materiales que hicieron posibles las sociedades guerreras. Hasta el momento, los estudios acerca del armamento son muchos y muy importantes; sin embargo, considero fundamental someter la «historia del armamento» al método de la historia social. Y aquí reside una de las diferencias fundamentales entre nuestra investigación y los demás estudios acerca del armamento. No se le concederá al armamento un valor autónomo y, por tanto, no será estudiado desde una perspectiva fenomenológica, sino que, por el contrario, se analizará en función de la sociedad. En consecuencia, se introducirá dentro de un período dotado de una homogeneidad social, que, en nuestro caso, será la sociedad feudal de los siglos XI al XIII.

  2. Venezuela-MEM/USA-DOE Fossil Energy Report XIII-1, Supporting Technology for Enhanced Oil Recovery, Microbial EOR

    Energy Technology Data Exchange (ETDEWEB)

    Ziritt, Jose Luis

    1999-11-03

    The results from Annex XIII of the Cooperative Agreement between the United States Department of Energy (DOE) and the Ministry of Energy and Mines of the Republic of Venezuela (MEMV) have been documented and published with many researchers involved. Integrate comprehensive research programs in the area of Microbial Enhanced Oil Recovery (MEOR) ranged from feasibility laboratory studies to full-scale multi-well field pilots. The objective, to cooperate in a technical exchange of ideas and information was fully met throughout the life of the Annex. Information has been exchanged between the two countries through published reports and technical meetings between experts in both country's research communities. The meetings occurred every two years in locations coincident with the International MEOR conferences & workshops sponsored by DOE (June 1990, University of Oklahoma, September 1992, Brookhaven, September 1995, National Institute of Petroleum and Energy Research). Reports and publications produced during these years are listed in Appendix B. Several Annex managers have guided the exchange through the years. They included Luis Vierma, Jose Luis Zirritt, representing MEMV and E. B. Nuckols, Edith Allison, and Rhonda Lindsey, representing the U.S. DOE. Funding for this area of research remained steady for a few years but decreased in recent years. Because both countries have reduced research programs in this area, future exchanges on this topic will occur through ANNEX XV. Informal networks established between researchers through the years should continue to function between individuals in the two countries.

  3. Electron-ion recombination measurements motivated by AGN X-ray absorption features: Fe XIV forming Fe XIII

    CERN Document Server

    Schmidt, E W; Müller, A; Lestinsky, M; Sprenger, F; Grieser, M; Repnow, R; Wolf, A; Brandau, C; Lukic, D; Schnell, M; Savin, D W

    2006-01-01

    Recent spectroscopic models of active galactic nuclei (AGN) have indicated that the recommended electron-ion recombination rate coefficients for iron ions with partially filled M-shells are incorrect in the temperature range where these ions form in photoionized plasmas. We have investigated this experimentally for Fe XIV forming Fe XIII. The recombination rate coefficient was measured employing the electron-ion merged beams method at the Heidelberg heavy-ion storage-ring TSR. The measured energy range of 0-260 eV encompassed all dielectronic recombination (DR) 1s2 2s2 2p6 3l 3l' 3l'' nl''' resonances associated with the 3p1/2 -> 3p3/2, 3s -> 3p, 3p -> 3d and 3s -> 3d core excitations within the M-shell of the Fe XIV 1s2 2s2 2p6 3s2 3p parent ion. This range also includes the 1s2 2s2 2p6 3l 3l' 4l'' nl''' resonances associated with 3s -> 4l'' and 3p -> 4l'' core excitations. We find that in the temperature range 2--14 eV, where Fe XIV is expected to form in a photoionized plasma, the Fe XIV recombination rate...

  4. Iron-Deficiency Anemia

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, ... Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by ...

  5. Iron-Deficiency Anemia

    Science.gov (United States)

    ... the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily ... Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the ...

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily ... Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the ...

  7. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily ... Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the ...

  8. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Deficiency Anemia? Español Iron-deficiency anemia is a common, easily treated condition that occurs if you don' ... from food. Overview Iron-deficiency anemia is a common type of anemia . The term "anemia" usually refers ...

  9. Factor XIII-A Val34Leu polymorphism might beassociated with myocardial infarction risk: an updated meta-analysis

    Science.gov (United States)

    Wang, Guangyun; Zou, Zhikang; Ji, Xiucai; Ni, Qingshan; Ma, Zhongli

    2014-01-01

    Although many epidemiologic studies have investigated the FXIII-A Val34Leu polymorphism and their associations with myocardial infarction (MI), definite conclusions can’t be drawn. To clarify the effects of FXIII-A Val34Leu polymorphism on the risk of MI, a meta-analysis was performed. Related studies were identified from PubMed, Springer Link, Ovid, Chinese Wanfang Data Knowledge Service Platform, Chinese National Knowledge Infrastructure (CNKI), and Chinese Biology Medicine (CBM) till 10 August 2014. Pooled ORs and 95% CIs were used to assess the strength of the associations. A total of 12 studies including 3139 MI cases and 6343 healthy controls were involved in this meta-analysis. A significantly decreased MI risk was found (adjusted OR = 0.70, 95% CI 0.60-0.82, P < 0.00001). In the subgroup analysis by age, significantly decreased risks were found in the young population (OR = 0.70, 95% CI 0.54-0.91, P = 0.008) and old population (OR = 0.63, 95% CI 0.50-0.80, P = 0.0001). In the subgroup analysis by gender, significantly decreased risks were found in male (OR = 0.55, 95% CI 0.34-0.88, P = 0.01) and female (OR = 0.72, 95% CI 0.55-0.95, P = 0.02). When we limited the meta-analysis to studies that controlled for confounders such as age, sex, BMI, smoking, diabetes, hypertension, dyslipidemia, and fibrinogen, a significant association between FXIII-A Val34Leu polymorphism and MI risk remained. This meta-analysis provides the evidence that FXIII-A Val34Leu polymorphism may significant associated with the MI risk. PMID:25664069

  10. Iron deficiency in blood donors

    Directory of Open Access Journals (Sweden)

    Armando Cortés

    2005-03-01

    Full Text Available Context: Blood donation results in a substantial loss of iron (200 to 250 mg at each bleeding procedure (425 to 475 ml and subsequent mobilization of iron from body stores. Recent reports have shown that body iron reserves generally are small and iron depletion is more frequent in blood donors than in non-donors. Objective: The aim of this study was to evaluate the frequency of iron deficiency in blood donors and to establish the frequency of iron deficiency in blood donors according to sex, whether they were first-time or multi-time donors. Design: From march 20 to April 5, 2004, three hundred potential blood donors from Hemocentro del Café y Tolima Grande were studied. Diagnostic tests: Using a combination of biochemical measurements of iron status: serum ferritin (RIA, ANNAR and the hemoglobin pre and post-donation (HEMOCUE Vital technology medical . Results: The frequency of iron deficiency in potential blood donors was 5%, and blood donors accepted was 5.1%; in blood donors rejected for low hemoglobin the frequency of iron deficiency was 3.7% and accepted blood donors was 1.7% in male and 12.6% in female. The frequency of iron deficiency was higher in multi-time blood donors than in first-time blood donors, but not stadistic significative. Increase nivel accepted hemoglobina in 1 g/dl no incidence in male; in female increase of 0.5 g/dl low in 25% blood donors accepted with iron deficiency, but increased rejected innecesary in 16.6% and increased is 1 g/dl low blood donors female accepted in 58% (7/12, but increased the rejected innecesary in 35.6%. Conclusions: We conclude that blood donation not is a important factor for iron deficiency in blood donors. The high frequency of blood donors with iron deficiency found in this study suggests a need for a more accurate laboratory trial, as hemoglobin or hematocrit measurement alone is not sufficient for detecting and excluding blood donors with iron deficiency without anemia, and ajustes hacia

  11. Combined effect of the environmental factors as ionizing radiation and a chronic iodine deficiency on the thyroid gland and the immune condition

    Energy Technology Data Exchange (ETDEWEB)

    Danyarova, L. [Department of Endocrynology, Research Institute of Cardiology and Internal Medicine, Almaty (Kazakhstan)

    2012-07-01

    The Semipalatinsk Test Site was the primary testing venue for the Soviet Union's nuclear weapons. It is located on the steppe in northeast Kazakhstan. The tragic situation of the Semipalatinsk region is an acute and chronic radiation, repeated in big and small doses and a total absence of territorial decontamination, created unique conditions for study of the long term influence of the radiation doses on the health of the population. The Semipalatinsk region of the Republic of Kazakhstan belongs also to an area of moderate and pronounced iodine deficiency. The purpose of the research is to study the prevalence of a thyroid gland pathology and the condition of a cytokine immune link that is likely to be influenced by a combine effect of ionizing radiation and a chronic iodine deficiency. 1100 people passed through the investigation and it appears that 56, 75% of them had a thyroid pathology. Thyroid gland functional condition analysis (TSH, FT3, FT4 a-TG, a-TPO) has shown the prevalence of a subclinical hypothyroidism (33%). 28, 8% resulted in the presence of antibodies to thyroglobulin and the thyroid peroxides, whereas in the areas located further to the nuclear range, the percentage was only 13, 0%

  12. Carnitine Deficiency and Pregnancy

    Directory of Open Access Journals (Sweden)

    Anouk de Bruyn

    2015-01-01

    Full Text Available We present two cases of carnitine deficiency in pregnancy. In our first case, systematic screening revealed L-carnitine deficiency in the first born of an asymptomatic mother. In the course of her second pregnancy, maternal carnitine levels showed a deficiency as well. In a second case, a mother known with carnitine deficiency under supplementation was followed throughout her pregnancy. Both pregnancies had an uneventful outcome. Because carnitine deficiency can have serious complications, supplementation with carnitine is advised. This supplementation should be continued throughout pregnancy according to plasma concentrations.

  13. The Analysis of Clinical Features and Risk Factors of Children's Nutritional Iron Deficiency Anemia%儿童营养性缺铁性贫血的临床特征及相关危险因素分析

    Institute of Scientific and Technical Information of China (English)

    徐丽琼

    2015-01-01

    目的:观察儿童营养性缺铁性贫血的临床表现症状并分析患病的相关危险因素。方法搜集2012年10月至2014年10月经我院确诊的288例营养性缺铁性贫血患儿资料,回顾性总结他们的临床表现特征,并分析造成儿童营养性缺铁性贫血的危险因素。结果288例营养性缺铁性贫血患儿,轻度贫血146例(50.69%),中度贫血113例(39.24%),重度贫血29例(10.07%),结果显示,年龄、孕周、是否接受育儿教育、是否为儿童添加辅食、儿童生活环境是否良好或稳定,是儿童患营养性缺铁性贫血的危险因素,结果具有统计学意义(P<0.05)。结论加强育儿知识教育和对小儿进行科学、合理的喂养,确保小儿良好、稳定的生活环境,是预防小儿营养性缺铁性贫血的有力措施。%Objective To observe the clinical features of children's nutritional iron deficiency anemia, and to analyze the risk factors.Methods The information of 288 cases confirmed nutritional deficiency anemia from October 2012 to October 2014 in our hospital were collected, and their clinical features were retrospectively summarized, and the risk factors of children's nutritional iron deficiency anemia were analyzed.Results Among the 288 children with nutritional iron deficiency anemia, mild anemia happened in 146 cases (50.69%), moderate anemia in 113 cases (39.24%) and severe anemia in 29 cases (10.07%).The results showed:Age, gestational weeks, whether to accept the parenting education, whether to add a side dish for children, and children's living en-vironment were the risk factors for children to suffer from nutritional iron deficiency anemia, and the results had statistical significance (P<0.05).Conclusion Strengthening the child rearing knowledge education, the scientific and rational feeding of children, and ensuring children to have good and stable living environment are effective measures for the

  14. [Vitamin A deficiency and xerophtalmia

    Science.gov (United States)

    Diniz, A da S; Santos, L M

    2000-11-01

    OBJECTIVE: To review cases of vitamin A deficiency and the effects of vitamin A supplementation on child morbidity and mortality. METHODS: Articles published in scientific journals, technical and scientific books, and also publications by international organizations were used as source of information. RESULTS: Clinical manifestations of xerophthalmia affect the retina (night blindness), the conjunctiva (conjunctival xerosis, with or without Bitot spots), and the cornea (corneal xerosis). Corneal xerosis may lead to corneal ulceration and liquefactive necrosis (keratomalacia). A priori, these signs and symptoms are the best indicators of vitamin A deficiency; they are, however, extremely rare. Laboratory indicators include Conjunctival Impression Cytology and serum retinol concentrations. The World Health Organization (WHO) recommends the use of two biological markers in order to characterize vitamin A deficiency in a given population. If only one biological marker is used, this marker has to be backed up by a set of at least four additional risk factors. Corneal xerophthalmia should be treated as a medical emergency; In the event of suspected vitamin A deficiency, a 200,000 IU vitamin A dose should be administered orally, repeating the dose after 24 hours (half the dose for infants younger than one year). Vitamin A supplementation in endemic areas may cause a 23 to 30% reduction in the mortality rate of children aged between 6 months and five years, and attenuate the severity of diarrhea. The methods for the control of vitamin A deficiency are available in the short (supplementation with megadoses), medium (food fortification), and long run (diet diversification). CONCLUSION: There is evidence of vitamin A deficiency among Brazilian children. Pediatricians must be aware of the signs and symptoms of this disease, however sporadic they might be. It is of paramount importance that vitamin A be included in public policy plans so that we can ensure the survival of

  15. Plasma ferritin and soluble transferrin receptor concentrations and body iron stores identify similar risk factors for iron deficiency but result in different estimates of the national prevalence of iron deficiency and iron-deficiency anemia among women and children in Cameroon.

    Science.gov (United States)

    Engle-Stone, Reina; Nankap, Martin; Ndjebayi, Alex O; Erhardt, Juergen G; Brown, Kenneth H

    2013-03-01

    Available iron status indicators reflect different aspects of metabolism. We compared the prevalence and distribution of iron deficiency (ID) and iron-deficiency anemia (IDA) among Cameroonian women and children, as measured by plasma ferritin, and soluble transferrin receptor concentrations, body iron stores (BIS), and hemoglobin, and evaluated the impact of adjustments for inflammation on these measures. In a nationally representative survey, we randomly selected 30 clusters in each of 3 zones (north, south, and large cities) and 10 households/ cluster, each with a child aged 12-59 mo and a woman 15-49 y. Ferritin and BIS were mathematically adjusted for inflammation, using plasma C-reactive protein and α(1)-acid glycoprotein both as continuous and categorical variables. Inflammation was present in 48.0% of children and 20.8% of women and anemia was diagnosed in 57.6% of children and 38.8% of women. Depending on the iron status indicator applied, the prevalence of ID ranged from 14.2 to 68.4% among children and 11.5 to 31.8% among women, and the prevalence of IDA ranged from 12.0 to 47.4% among children and 9.0 to 19.4% among women; the proportion of anemia associated with ID ranged from 20.8 to 82.3% among children and 23.2 to 50.0% among women. The different iron indicators generally identified similar groups at greatest risk of deficiency, using both conventional and derived cutoffs: younger children, pregnant women, and women and children in the north and rural areas. Research is needed to clarify the relationships between iron status indicators, particularly in the presence of inflammation, to harmonize global data on prevalence of ID.

  16. 深圳市综合医院护患沟通障碍原因调查研究%A Survey on factors of nurse-patient communication deficiencies in municipal hospitals in Shenzhen

    Institute of Scientific and Technical Information of China (English)

    雷志荣; 刘伟; 刘璞娉; 麦爱欢; 夏春梅; 张小燕

    2013-01-01

    Objective To explore factors of nurse-patient communication deficiencies in municipal hospitals in Shenzhen.Methods A total of 147 inpatients and 146 nurses from 8 municipal teaching hospitals were enrolled to complete a questionnaire referred to Anoosheh's.All the participants were asked to give marks to every communication barriers according to the questionnaire.Data were collected and analyzed with statistical software SSPS 17.0.Results The results shows that the marks given by the patients and by the nurses were different significantly.Variance analysis results performed on the four main factors listed in the questionnaire (factors on administration,factors on nurse,factors on patient and factors on social/circumstance/gender and others) revealed that the role of this four factors on nurse-patient communication deficiencies was remarkably different with which factors on administration had the most important role and factors on nurse had the least important role.Logisc regression analysis showed that the first three subdivision factors of the factors on administration were" too many critical patients to take care and too many rescues in the nurse's work,the nurse has to confront overload stress","shortage of nursing staff in the hospital" and" the nurse has to spent too much time on the cure work ".Conclusions Nurses and patients may have different point of view to the same thing when they are communicating,so the nurse should try to reduce the gap of this difference.Factors on administration are the main cause of nurse-patient communication deficiency which the nursing managers and administrators should pay attention to and try to improve administrating level to relive nurse's stress and reduce the workload.%目的 研究深圳市综合医院护患沟通障碍的原因.方法 参照伊朗Anoosheh硕士的调查问卷,稍作改动,对深圳8个综合医院147名住院患者和146名护士进行调查,参加者被要求对问卷上的沟通障碍各原因进行

  17. Materiales y técnicas constructivas en la Murcia andalusí (siglos X-XIII

    Directory of Open Access Journals (Sweden)

    Navarro Palazón, Julio

    2011-12-01

    Full Text Available The evolution of the materials and building techniques of the andalusian city of Murcia between the 9th and 13th centuries are here studied, mainly based on the new information provided by the numerous archaeological excavations that have been carried out in the city for the last thirty years, related especially to residential architecture. After its accuracy analysis, some differences between the buildings techniques manufactured in the 10th and 11th centuries and those later attributed to 12th and 13th centuries have been identified, being thus highly important to describe them by remarking at the same time those distinguishing features. Problems related to the absolute chronology of these building techniques are also discussed, highlighting the definitive information currently known. With the main aim of analysing how general these remarks are or, on the contrary, if they depend on the regionalization phenomenon characterizing the andalusian recording material, we will compare the information obtained in Murcia with those coming from other andalusian sites, located these far away from the studied area. Finally, we will check if there are differences between the rural and urban environments by comparing the materials and building techniques recorded in two coetaneous sites in Murcia, both sited close to the same river Segura and 40 km distant from each other: the city of Murcia (a large madîna and the deserted settlement of Siyâsa (a hisn.En el presente trabajo estudiamos la evolución de los materiales y técnicas constructivas en la Murcia andalusí, durante un periodo de tiempo que se extiende entre los siglos X y XIII, para lo que nos basaremos especialmente en la rica información extraída de las numerosas excavaciones efectuadas en dicha ciudad, que se refiere sobre todo a arquitectura residencial. Tras su exhaustivo análisis hemos identificado algunas diferencias entre las fábricas de los siglos X y XI y las posteriores de los

  18. O trovadorismo medieval ibérico e a violência simbólica - séculos XIII e XIV

    Directory of Open Access Journals (Sweden)

    Barros, José D´Assunção

    2006-01-01

    Full Text Available O objetivo deste artigo é discutir a violência simbólica presente nas sociedades medievais ibéricas dos séculos XIII e XIV, examinando a poesia e práticas trovadorescas do período. Dentro do contexto histórico da centralização régia do período medieval, o texto analisa algumas cantigas trovadorescas galego-portuguesas nas quais podem ser vistas as tensões sociais entre trovadores oriundos de diferentes categorias sociais, e também envolvendo trovadores em oposição ao rei

  19. Un nuevo ejemplar ilustrado de los Morales de Gregorio Magno (B.A.H. COD. 1 del siglo XIII, inédito

    Directory of Open Access Journals (Sweden)

    de Silva y Verastegui, Soledad

    1996-12-01

    Full Text Available The codex was copied and illustrated in the early decades of the XIII century, in the scriptorium of San Millan de la Cogolla. This scriptorium was at this time going through a renewed age of splendour which fully coincided in time with the intense literary work carried out by Gonzalo de Berceo. In this paper we analyse the miniatures and decorative initials from an iconography and a stylistic perspective, and we identify its authors as being the same artists who illustrated the celebrated Biblia de San Millan (B.A.H. cod. 2 and 3.No disponible

  20. Jurisdictional conflict and writing competition between the cathedral of León and the monastery of Sahagún: the production of Becerros in the XIII Century

    Directory of Open Access Journals (Sweden)

    Leticia Agúndez San Miguel

    2016-12-01

    Full Text Available This article examines the consequences of the production of the Becerro de Presentaciones by the scriptorium of the Cathedral of León and the Becerro Segundo by the monastery of Sahagún in the last stage of the long jurisdictional conflict between these two institutions. Its production not only allows us to appreciate the writing competition that existed between them because of their controversial relationship but also inform us about the emergence of a new social dynamic related to the function of writing in the XIII century.

  1. O Diabo e suas representações simbólicas em Ramon Llull e Dante Alighieri (séculos XIII e XIV)

    OpenAIRE

    Loureiro, Klítia

    2002-01-01

    Este artigo visa recuperar os elementos fundamentais da concepção de Diabo e de Inferno para os medievos, particularmente durante os séculos XIII e XIV. Analisamos a visão de Diabo e de Inferno descrita por Ramon Llull (1232-1316) no Livro das Maravilhas (1288-1289), na Doutrina para Crianças (1274-1276) e no Livro dos Anjos (1274?-1283?), comparando brevemente sua concepção com a de Dante Alighieri na Divina Comédia (1307-1321). This article intents to recover the fundamentals elements of...

  2. Paleopatología de la columna vertebral en la población hispanomusulmana de San Nicolás, (Murcia, S. XI-XIII)

    OpenAIRE

    Campo Martín, Manuel

    2016-01-01

    El objetivo general de esta tesis doctoral es profundizar en nuestros conocimientos actuales acerca de una estructura del esqueleto, fundamental en nuestra filogenia, y aún sujeta a múltiples interrogantes: el raquis o columna vertebral. Para nuestra investigación hemos utilizado como muestra de estudio una población arqueológica hispanomusulmana de época medieval (San Nicolás. Murcia, s. XI-XIII), tratando de demostrar que la paleopatología, como disciplina científica dual historicomédica, t...

  3. «La obediencia constante y leal a la autoridad pública ». León XIII y el comienzo de la regencia de María Cristina

    OpenAIRE

    2006-01-01

    After the death of Alfonso XII a period of uncertainty was opened. The danger of a new civil war and of republican raisings they were threatening Maria Cristina’s regency. León XIII supported to Regent and asked to bishops, clergy and Spanish Catholics who were accepting the legality.Tras la muerte de Alfonso XII se abría un periodo de incertidumbre. El peligro de una nueva guerra civil y de levantamientos republicanos amenazaba la regencia de María Cristina. León XIII apoyó a la regente y pi...

  4. Matrix Metalloproteinase-2-deficient Fibroblasts Exhibit an Alteration in the Fibrotic Response to Connective Tissue Growth Factor/CCN2 because of an Increase in the Levels of Endogenous Fibronectin*

    Science.gov (United States)

    Droppelmann, Cristian A.; Gutiérrez, Jaime; Vial, Cecilia; Brandan, Enrique

    2009-01-01

    Matrix metalloproteinase-2 (MMP-2) is an important extracellular matrix remodeling enzyme, and it has been involved in different fibrotic disorders. The connective tissue growth factor (CTGF/CCN2), which is increased in these pathologies, induces the production of extracellular matrix proteins. To understand the fibrotic process observed in diverse pathologies, we analyzed the fibroblast response to CTGF when MMP-2 activity is inhibited. CTGF increased fibronectin (FN) amount, MMP-2 mRNA expression, and gelatinase activity in 3T3 cells. When MMP-2 activity was inhibited either by the metalloproteinase inhibitor GM-6001 or in MMP-2-deficient fibroblasts, an increase in the basal amount of FN together with a decrease of its levels in response to CTGF was observed. This paradoxical effect could be explained by the fact that the excess of FN could block the access to other ligands, such as CTGF, to integrins. This effect was emulated in fibroblasts by adding exogenous FN or RGDS peptides or using anti-integrin αV subunit-blocking antibodies. Additionally, in MMP-2-deficient cells CTGF did not induce the formation of stress fibers, focal adhesion sites, and ERK phosphorylation. Anti-integrin αV subunit-blocking antibodies inhibited ERK phosphorylation in control cells. Finally, in MMP-2-deficient cells, FN mRNA expression was not affected by CTGF, but degradation of 125I-FN was increased. These results suggest that expression, regulation, and activity of MMP-2 can play an important role in the initial steps of fibrosis and shows that FN levels can regulate the cellular response to CTGF. PMID:19276073

  5. O argumento da linhagem na literatura ibérica do séc. XIII

    Directory of Open Access Journals (Sweden)

    José Carlos Ribeiro MIRANDA

    2011-06-01

    Full Text Available Partindo da identificação e localização dos primeiros usos do termo «linhagem» e dos seus equivalentes nas várias línguas de raiz latina da Península Ibérica ao longo do séc. XIII, o presente estudo identifica dois envolvimentos semânticos que caracterizam esse uso, tanto em textos literários como documentais da mais variada tipologia: por um lado, um sentido que se refere à ascendência-descendência de um dado indivíduo, numa tradição que parece próxima da que se detecta no Liber Regum; por outro, um sentido que identifica «linhagem» com um grupo de parentes de sangue coexistentes num dado espaço, não sendo fácil identificar contextos discursivos em que ambas as acepções se combinem.Partant de l’identification des premiers usages du mot «linhagem» et de ses équivalents en langues ibériques néo-latines au long du XIIIe siècle, on repère dans des textes littéraires et documentaires de typologies diverses deux spécialisations sémantiques de ce terme. D’un côté, «linhagem» désigne l’ascendance-descendance d’un individu précis, dans une tradition qui semble proche de celle du Liber Regum; de l’autre côté, le même mot désigne un groupe synchronique de parents consanguins habitant dans la même région. La coexistence des deux acceptions dans un même contexte discursif est rare.    

  6. Un quirógrafo impugnado. Zamora y la cultura jurídica zamorana a comienzos del siglo XIII

    Directory of Open Access Journals (Sweden)

    Linehan, Peter

    2009-06-01

    Full Text Available Because so soon after it was overtaken by the School of Salamanca, historians have neglected the importance of the Chapter of nearby Zamora in the legal development of early thirtheenth century Spain. In the light of the ambivalent significance of Zamora’s proximity to the kingdom of Portugal in a period of political tension with the papacy, and the role of Zamora’s man at the papal curia, Cardinal Gil Torres, the present study attempts to view that development in terms of its own time, making use of the material evidence in the Vatican and peninsular archives of Zamora’s juristic activity, the level of juristic sophistication revealed by a routine case involving local clergy and laity, and the calculations of the contemporary rulers of León.

    Debido a que muy pronto fue sobrepasada por la Escuela de Salamanca, los historiadores han descuidado la importancia del Capítulo de la cercana Zamora en el progreso jurídico español de los comienzos del siglo XIII. A la luz del ambivalente significado de la proximidad de Zamora al reino de Portugal, en un periodo de tensión política con el papado y del papel desempeñado por el Cardenal zamorano Gil Torres en la curia papal, el presente estudio se propone examinar ese progreso de la actividad jurídica de Zamora durante su tiempo, utilizando para ello los testimonios de los archivos del Vaticano y peninsulares, el nivel de sofisticación jurídica manifestado en un caso rutinario en el que se implicaba un clérigo local y el laicado, así como las astucias de los gobernantes coetáneos de León.

  7. Esclavos musulmanes en los hospitales de cautivos de la Orden Militar de Santiago (siglos XII y XIII

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    Echevarría Arsuaga, Ana

    2007-12-01

    Full Text Available Military Orders had slave manpower in their lands since their foundation. However, the hospitals for ransoming captives owned by the Order of Santiago are the best example of how slavery worked in the kingdom of Leon-Castile during the twelfth and thirteenth centuries. These houses had enough room to provide lodging for captives while ransoms were being negotiated. They also helped to redistribute those Muslims who, lacking this possibility, became slaves of the order itself, or were sold in the slave markets of the kingdom. The scarce records kept in the Archivo Histórico Nacional, section Órdenes Militares, referring to slaves, help to understand the development of traffic, work and manumission of these people.

    Las órdenes militares dispusieron desde su fundación de mano de obra esclava en sus tierras, pero la institución que mejor sirve para estudiar la esclavitud en los siglos XII y XIII en los reinos de León y Castilla son los hospitales para la redención de cautivos de la Orden de Santiago. Estas instituciones disponían de espacio para alojar a los cautivos mientras se negociaban rescates con los poderes islámicos, y atendían a la redistribución de aquellos musulmanes que, por no haber sido redimidos, pasaban a ser esclavos de la propia orden o a ser vendidos en los mercados de los reinos cristianos. Los escasos documentos de la sección de Órdenes Militares del Archivo Histórico Nacional dedicados a esclavos ayudan a comprender el funcionamiento del tráfico, trabajo y liberación de este colectivo.

  8. Acquired color vision deficiency.

    Science.gov (United States)

    Simunovic, Matthew P

    2016-01-01

    Acquired color vision deficiency occurs as the result of ocular, neurologic, or systemic disease. A wide array of conditions may affect color vision, ranging from diseases of the ocular media through to pathology of the visual cortex. Traditionally, acquired color vision deficiency is considered a separate entity from congenital color vision deficiency, although emerging clinical and molecular genetic data would suggest a degree of overlap. We review the pathophysiology of acquired color vision deficiency, the data on its prevalence, theories for the preponderance of acquired S-mechanism (or tritan) deficiency, and discuss tests of color vision. We also briefly review the types of color vision deficiencies encountered in ocular disease, with an emphasis placed on larger or more detailed clinical investigations.

  9. Stimulation of the 150-kilodalton insulin-like growth factor-binding protein-3 ternary complex by continuous and pulsatile patterns of growth hormone (GH) administration in GH-deficient patients

    DEFF Research Database (Denmark)

    Laursen, Torben; Flyvbjerg, Allan; Jørgensen, Jens Otto Lunde

    2000-01-01

    Abstract In the circulation insulin-like growth factor I (IGF-I), IGF-binding protein 3 (IGFBP-3), and the acid-labile subunit (ALS) form a 150-kDa ternary complex that is of importance for the regulation of IGF-I bioactivity. GH administration is known to increase each of the single components...... of the ternary complex, and in GH-deficient rats formation of the 150-kDa complex is induced more by continuous than by pulsatile GH patterns. The aim of the present studies was to study the effects of the GH administration pattern on the formation of the 150-kDa ternary complex in humans. A fixed total GH dose...... (2 IU/m2-24 h) was administered iv randomly as 1) continuous infusion or 2) eight bolus injections to five GH-deficient patients over a period of 24 h. GH administration significantly increased serum IGF-I and IGFBP-3 levels and the IGF-I/IGFBP-3 ratio. IGF-I levels increased most pronouncedly after...

  10. Growth hormone (GH)-transgenic insulin-like growth factor 1 (IGF1)-deficient mice allow dissociation of excess GH and IGF1 effects on glomerular and tubular growth.

    Science.gov (United States)

    Blutke, Andreas; Schneider, Marlon R; Wolf, Eckhard; Wanke, Rüdiger

    2016-03-01

    Growth hormone (GH)-transgenic mice with permanently elevated systemic levels of GH and insulin-like growth factor 1 (IGF1) reproducibly develop renal and glomerular hypertrophy and subsequent progressive glomerulosclerosis, finally leading to terminal renal failure. To dissociate IGF1-dependent and -independent effects of GH excess on renal growth and lesion development in vivo, the kidneys of 75 days old IGF1-deficient (I(-/-)) and of IGF1-deficient GH-transgenic mice (I(-/-)/G), as well as of GH-transgenic (G) and nontransgenic wild-type control mice (I(+/+)) were examined by quantitative stereological and functional analyses. Both G and I(-/-)/G mice developed glomerular hypertrophy, hyperplasia of glomerular mesangial and endothelial cells, podocyte hypertrophy and foot process effacement, albuminuria, and glomerulosclerosis. However, I(-/-)/G mice exhibited less severe glomerular alterations, as compared to G mice. Compared to I(+/+) mice, G mice exhibited renal hypertrophy with a significant increase in the number without a change in the size of proximal tubular epithelial (PTE) cells. In contrast, I(-/-)/G mice did not display significant PTE cell hyperplasia, as compared to I(-/-) mice. These findings indicate that GH excess stimulates glomerular growth and induces lesions progressing to glomerulosclerosis in the absence of IGF1. In contrast, IGF1 represents an important mediator of GH-dependent proximal tubular growth in GH-transgenic mice.

  11. Severe Vitamin D Deficiency Causing Kyphoscoliosis.

    Science.gov (United States)

    Singhai, Abhishek; Banzal, Subodh

    2013-01-01

    Vitamin D deficiency is common among Indian population. Women are especially at risk for severe vitamin D deficiency. The risk is higher for those who are multiparous and postmenopausal. Poor exposure to sunlight, higher latitude, winter season, inadequate diet, older age, obesity and malabsorption are also important risk factors. Symptoms of hypovitaminosis D, including diffuse or migratory pain affecting several sites (especially the shoulder, pelvis, ribcage and lower back) have also been misdiagnosed as musculoskeletal disorders, including fibromyalgia, polymyalgia rheumatica and ankylosing spondylitis. Here, we report two cases presented with kyphoscoliosis, diagnosed to have severe vitamin D deficiency.

  12. Selective deletion of the membrane-bound colony stimulating factor 1 isoform leads to high bone mass but does not protect against estrogen-deficiency bone loss.

    Science.gov (United States)

    Yao, Gang-Qing; Wu, Jian-Jun; Troiano, Nancy; Zhu, Mei-Ling; Xiao, Xiao-Yan; Insogna, Karl

    2012-07-01

    To better define the biologic function of membrane-bound CSF1 (mCSF1) in vivo, we have generated mCSF1 knockout (k/o) mice. Spinal bone density (BMD) was 15.9% higher in k/o mice compared to wild-type (wt) controls (P bone marrow isolated from mCSF1 k/o mice was reduced compared to wt marrow. There were no defects in osteoblast number or function suggesting that the basis for the high bone mass phenotype was reduced resorption. In addition to a skeletal phenotype, k/o mice had significantly elevated serum triglyceride levels (123 ± 7 vs. 88 ± 3.2 mg/dl; k/o vs. wt, P bone loss following ovariectomy (OVX). OVX induced a significant fourfold increase in the expression of the soluble CSF1 isoform (sCSF1) in the bones of wt mice while expression of mCSF1 was unchanged. These findings indicate that mCSF1 is essential for normal bone remodeling since, in its absence, BMD is increased. Membrane-bound CSF1 does not appear to be required for estrogen-deficiency bone loss while in contrast; our data suggest that sCSF1 could play a key role in this pathologic process. The reasons why mCSF1 k/o mice have hypertriglyceridemia are currently under study.

  13. A review of iron deficiency anemia to diet factor%膳食因素与缺铁性贫血的研究进展

    Institute of Scientific and Technical Information of China (English)

    潘炜; 袁宝君

    2010-01-01

    @@ 缺铁性贫血(iron deficiency anemia,IDA)是指体内用来制造血红蛋白的贮存铁耗尽,红细胞生成受到障碍时所发生的贫血,是铁缺乏症(ID)的晚期表现.全球有66%~80%的人口处于铁缺乏状态,其中一半以上为缺铁性贫血.铁缺乏症和缺铁性贫血严重影响了各年龄层次人群的精神状态、生理活动及工作能力,使机体抵抗力下降,尤其对儿童、妇女和老年人的危害较为严重 [1].目前认为,低铁生物利用率是导致IDA和ID的主要原因之一,膳食因素可显著影响铁的生物利用率.

  14. Environmental controls on iodine deficiency disorders (IDD)

    OpenAIRE

    Johnson, C C; Fordyce, F.M.

    2003-01-01

    It is estimated that in excess of one billion people world-wide are at risk from iodine deficiency disorders (IDD), the most obvious manifestation of which is goitre (see Fordyce, 2000). Iodine deficiency is the world’s most common cause of mental retardation and brain damage, and the negative effects of impaired mental function have a significant impact on the social and economic development of communities. Although IDD can be caused by a number of factors, goitrogens fo...

  15. Severe Vitamin D Deficiency Causing Kyphoscoliosis

    OpenAIRE

    Singhai, Abhishek; Banzal, Subodh

    2013-01-01

    Vitamin D deficiency is common among Indian population. Women are especially at risk for severe vitamin D deficiency. The risk is higher for those who are multiparous and postmenopausal. Poor exposure to sunlight, higher latitude, winter season, inadequate diet, older age, obesity and malabsorption are also important risk factors. Symptoms of hypovitaminosis D, including diffuse or migratory pain affecting several sites (especially the shoulder, pelvis, ribcage and lower back) have also been ...

  16. Cobalamin deficiency, hyperhomocysteinemia, and dementia

    Directory of Open Access Journals (Sweden)

    Steven F Werder

    2010-04-01

    Full Text Available Steven F Werder1,21Kansas University School of Medicine – Wichita, Wichita, KS, USA; 2Community Health Center of Southeast Kansas, Pittsburg, KS, USAIntroduction: Although consensus guidelines recommend checking serum B12 in patients with dementia, clinicians are often faced with various questions: (1 Which patients should be tested? (2 What test should be ordered? (3 How are inferences made from such testing? (4 In addition to serum B12, should other tests be ordered? (5 Is B12 deficiency compatible with dementia of the Alzheimer’s type? (6 What is to be expected from treatment? (7 How is B12 deficiency treated?Methods: On January 31st, 2009, a Medline search was performed revealing 1,627 citations related to cobalamin deficiency, hyperhomocysteinemia, and dementia. After limiting the search terms, all abstracts and/or articles and other references were categorized into six major groups (general, biochemistry, manifestations, associations and risks, evaluation, and treatment and then reviewed in answering the above questions.Results: The six major groups above are described in detail. Seventy-five key studies, series, and clinical trials were identified. Evidence-based suggestions for patient management were developed.Discussion: Evidence is convincing that hyperhomocysteinemia, with or without hypovitaminosis B12, is a risk factor for dementia. In the absence of hyperhomocysteinemia, evidence is less convincing that hypovitaminosis B12 is a risk factor for dementia. B12 deficiency manifestations are variable and include abnormal psychiatric, neurological, gastrointestinal, and hematological findings. Radiological images of individuals with hyperhomocysteinemia frequently demonstrate leukoaraiosis. Assessing serum B12 and treatment of B12 deficiency is crucial for those cases in which pernicious anemia is suspected and may be useful for mild cognitive impairment and mild to moderate dementia. The serum B12 level is the standard initial test

  17. Serum insulin-like factor 3 is highly correlated with intratesticular testosterone in normal men with acute, experimental gonadotropin deficiency stimulated with low-dose human chorionic gonadotropin

    DEFF Research Database (Denmark)

    Roth, Mara Y; Lin, Kat; Bay, Katrine

    2013-01-01

    To study the potential role for using serum biomarkers, including insulin-like factor 3 (INSL3), 17α-hydroxyprogesterone, antimüllerian hormone, and inhibin B, as correlates of intratesticular T (IT-T) concentrations in men....

  18. Double heterozygous mutations Gln100Leu and His348Gln of the F7 gene in a patient with factor VII deficiency.

    Science.gov (United States)

    Li, Min; Zheng, Fangxiu; Jin, Yanhui; Wang, Mingshan; Zhu, Liqing; Yang, Lihong

    2013-03-01

    A 25-year-old Chinese woman who had a history of easy bruising was admitted to hospital due to uncontrolled epistaxis. She showed factor VII activity level of 2% and factor VII antigen level of 4% of the normal value. We detected a novel missense mutation g.8355 A>T (p.Gln100Leu) in the second epidermal growth factor-like (EGF) domain and a g.11482 T>G (p.His348Gln) in the catalytic domain. Although the Gln100 residue is close to the junction of EGF-2 domain with the serine protease domain, we infer that the substitution of polar negatively charged Gln residue at the position 100 with introduction of nonpolar Leu residue may be likely to perturb proper folding, resulting in decreasing factor VII activity.

  19. Adipocyte-Specific Hypoxia-Inducible FactorDeficiency Exacerbates Obesity-Induced Brown Adipose Tissue Dysfunction and Metabolic Dysregulation

    OpenAIRE

    García-Martín, Rubén; Alexaki, Vasileia I.; Qin, Nan; Rubín de Celis, María F.; Economopoulou, Matina; Ziogas, Athanasios; Gercken, Bettina; Kotlabova, Klara; Phieler, Julia; Ehrhart-Bornstein, Monika; Bornstein, Stefan R.; Eisenhofer, Graeme; BREIER, GEORG; Blüher, Matthias; Hampe, Jochen

    2016-01-01

    Angiogenesis is a central regulator for white (WAT) and brown (BAT) adipose tissue adaptation in the course of obesity. Here we show that deletion of hypoxia-inducible factor 2α (HIF2α) in adipocytes (by using Fabp4-Cre transgenic mice) but not in myeloid or endothelial cells negatively impacted WAT angiogenesis and promoted WAT inflammation, WAT dysfunction, hepatosteatosis, and systemic insulin resistance in obesity. Importantly, adipocyte HIF2α regulated vascular endothelial growth factor ...

  20. Initiation of liver growth by tumor necrosis factor: Deficient liver regeneration in mice lacking type I tumor necrosis factor receptor

    OpenAIRE

    Yamada, Yasuhiro; Kirillova, Irina; Peschon, Jacques J.; Fausto, Nelson

    1997-01-01

    The mechanisms that initiate liver regeneration after resection of liver tissue are not known. To determine whether cytokines are involved in the initiation of liver growth, we studied the regeneration of the liver after partial hepatectomy (PH) in mice lacking type I tumor necrosis factor receptor (TNFR-I). DNA synthesis after PH was severely impaired in these animals, and the expected increases in the binding of the NF-κB and STAT3 transcription factors shortly after...

  1. 'Nizovata' knižnina prez XIII vek: Sbornici, proizvedenija i cikli v b'lgarskata i v sr'bskata tradicija

    Directory of Open Access Journals (Sweden)

    Miltenova Anisava

    2009-01-01

    Full Text Available (bugarski V statijata se razgleždat v sravnitelen plan sbornici ot XIII i ot XIV v. ot sr'bskata i ot b'lgarskata r'kopisna tradicija, v koito preobladavat apokrifi. Očertavat se tendenciite za preantologizirane na proizvedenija i cikli ot 'nizovata' knižnina, s'zdadeni prez po-rannite epohi (X v. i osobeno XI-XII v i s'zdavaneto na novi kompilativni, prevodni i originalni tvorbi. Specialno mjasto se otdelja na istoriko-apokaliptičnite s'činenija, aktualni za XIII v. Sr'bskite i b'lgarskite r'kopisi svidetelstvat, če v prod'lž enie na vekove apokrifite sa se vključvali sred dušepoleznite četiva za izv'nbogoslužebna upotreba, bez da se pravi razlika meždu kanonično i nekanonič no. T. nar. parabiblejska literatura (v obščohristijanski aspekt, pritež ava za balkanskite knižovnici ne po-malko značenie i dostojnstvo ot učitelnata hristijanska literatura.

  2. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Blood Tests Blood Transfusion Restless Legs Syndrome Send a link to NHLBI to someone by E-MAIL | ... Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily treated condition that occurs if you ...

  3. Iron deficiency anemia

    Science.gov (United States)

    Anemia - iron deficiency ... iron from old red blood cells. Iron deficiency anemia develops when your body's iron stores run low. ... You may have no symptoms if the anemia is mild. Most of the time, ... slowly. Symptoms may include: Feeling weak or tired more often ...

  4. Muscle phosphorylase kinase deficiency

    DEFF Research Database (Denmark)

    Preisler, N; Orngreen, M C; Echaniz-Laguna, A;

    2012-01-01

    To examine metabolism during exercise in 2 patients with muscle phosphorylase kinase (PHK) deficiency and to further define the phenotype of this rare glycogen storage disease (GSD).......To examine metabolism during exercise in 2 patients with muscle phosphorylase kinase (PHK) deficiency and to further define the phenotype of this rare glycogen storage disease (GSD)....

  5. Growth Hormone Deficiency

    Directory of Open Access Journals (Sweden)

    Ömer Tarım

    2010-05-01

    Full Text Available Growth hormone deficiency is the most promising entity in terms of response to therapy among the treatable causes of growth retardation. It may be due to genetic or acquired causes. It may be isolated or a part of multiple hormone deficiencies. Diagnostic criteria and therefore treatment indications are still disputed. (Journal of Current Pediatrics 2010; 8: 36-8

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... periods. By following her treatment plan and making smart lifestyle choices, Susan continues to feel better and see the benefits of treatment. For more information about living with and managing iron-deficiency anemia, go to the Health Topics Iron-Deficiency Anemia article. Updated: March 26, ...

  7. Iron induced nickel deficiency

    Science.gov (United States)

    It is increasingly apparent that economic loss due to nickel (Ni) deficiency likely occurs in horticultural and agronomic crops. While most soils contain sufficient Ni to meet crop requirements, situations of Ni deficiency can arise due to antagonistic interactions with other metals. This study asse...

  8. Iron deficiency in childhood

    NARCIS (Netherlands)

    Uijterschout, L.

    2015-01-01

    Iron deficiency (ID) is the most common micronutrient deficiency in the world. Iron is involved in oxygen transport, energy metabolism, immune response, and plays an important role in brain development. In infancy, ID is associated with adverse effects on cognitive, motor, and behavioral development

  9. Deficiently Extremal Gorenstein Algebras

    Indian Academy of Sciences (India)

    Pavinder Singh

    2011-08-01

    The aim of this article is to study the homological properties of deficiently extremal Gorenstein algebras. We prove that if / is an odd deficiently extremal Gorenstein algebra with pure minimal free resolution, then the codimension of / must be odd. As an application, the structure of pure minimal free resolution of a nearly extremal Gorenstein algebra is obtained.

  10. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Blood Tests Blood Transfusion Restless Legs Syndrome Send a link to NHLBI to someone by E-MAIL | ... Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily treated condition that occurs if you ...

  11. Iron deficiency in childhood

    NARCIS (Netherlands)

    Uijterschout, L.

    2015-01-01

    Iron deficiency (ID) is the most common micronutrient deficiency in the world. Iron is involved in oxygen transport, energy metabolism, immune response, and plays an important role in brain development. In infancy, ID is associated with adverse effects on cognitive, motor, and behavioral development

  12. A sustained deficiency of mitochondrial respiratory complex III induces an apoptotic cell death through the p53-mediated inhibition of pro-survival activities of the activating transcription factor 4.

    Science.gov (United States)

    Evstafieva, A G; Garaeva, A A; Khutornenko, A A; Klepikova, A V; Logacheva, M D; Penin, A A; Novakovsky, G E; Kovaleva, I E; Chumakov, P M

    2014-11-06

    Generation of energy in mitochondria is subjected to physiological regulation at many levels, and its malfunction may result in mitochondrial diseases. Mitochondrial dysfunction is associated with different environmental influences or certain genetic conditions, and can be artificially induced by inhibitors acting at different steps of the mitochondrial electron transport chain (ETC). We found that a short-term (5 h) inhibition of ETC complex III with myxothiazol results in the phosphorylation of translation initiation factor eIF2α and upregulation of mRNA for the activating transcription factor 4 (ATF4) and several ATF4-regulated genes. The changes are characteristic for the adaptive integrated stress response (ISR), which is known to be triggered by unfolded proteins, nutrient and metabolic deficiency, and mitochondrial dysfunctions. However, after a prolonged incubation with myxothiazol (13-17 h), levels of ATF4 mRNA and ATF4-regulated transcripts were found substantially suppressed. The suppression was dependent on the p53 response, which is triggered by the impairment of the complex III-dependent de novo biosynthesis of pyrimidines by mitochondrial dihydroorotate dehydrogenase. The initial adaptive induction of ATF4/ISR acted to promote viability of cells by attenuating apoptosis. In contrast, the induction of p53 upon a sustained inhibition of ETC complex III produced a pro-apoptotic effect, which was additionally stimulated by the p53-mediated abrogation of the pro-survival activities of the ISR. Interestingly, a sustained inhibition of ETC complex I by piericidine did not induce the p53 response and stably maintained the pro-survival activation of ATF4/ISR. We conclude that a downregulation of mitochondrial ETC generally induces adaptive pro-survival responses, which are specifically abrogated by the suicidal p53 response triggered by the genetic risks of the pyrimidine nucleotide deficiency.

  13. DNA repair deficiency in neurodegeneration

    DEFF Research Database (Denmark)

    Jeppesen, Dennis Kjølhede; Bohr, Vilhelm A; Stevnsner, Tinna V.

    2011-01-01

    : homologous recombination and non-homologous end-joining. Ataxia telangiectasia and related disorders with defects in these pathways illustrate that such defects can lead to early childhood neurodegeneration. Aging is a risk factor for neurodegeneration and accumulation of oxidative mitochondrial DNA damage......Deficiency in repair of nuclear and mitochondrial DNA damage has been linked to several neurodegenerative disorders. Many recent experimental results indicate that the post-mitotic neurons are particularly prone to accumulation of unrepaired DNA lesions potentially leading to progressive...... neurodegeneration. Nucleotide excision repair is the cellular pathway responsible for removing helix-distorting DNA damage and deficiency in such repair is found in a number of diseases with neurodegenerative phenotypes, including Xeroderma Pigmentosum and Cockayne syndrome. The main pathway for repairing oxidative...

  14. Treatment of zinc deficiency without zinc fortification

    Institute of Scientific and Technical Information of China (English)

    Donald OBERLEAS; Barbara F. HARLAND

    2008-01-01

    Zinc (Zn) deficiency in animals became of interest until the 1950s. In this paper, progresses in researches on physi-ology of Zn deficiency in animals, phytate effect on bioavailability of Zn, and role of phytase in healing Zn deficiency of animals were reviewed. Several studies demonstrated that Zn is recycled via the pancreas; the problem of Zn deficiency was controlled by Zn homeostasis. The endogenous secretion of Zn is considered as an important factor influencing Zn deficiency, and the critical molar ratio is 10. Phytate (inositol hexaphosphate) constituted up to 90% of the organically bound phosphorus in seeds. Great improvement has been made in recent years on isolating and measuring phytate, and its structure is clear. Phytate is considered to reduce Zn bioavailability in animal. Phytase is the enzyme that hydrolyzes phytate and is present in yeast, rye bran, wheat bran, barley, triticale, and many bacteria and fungi. Zinc nutrition and bioavailability can be enhanced by addition of phytase to animal feeds. Therefore, using phytase as supplements, the most prevalent Zn deficiency in animals may be effectively corrected without the mining and smelting of several tons of zinc daily needed to correct this deficiency by fortification worldwide.

  15. Serum free insulin-like growth factor-I in growth hormone-deficient adults before and after growth hormone replacement.

    Science.gov (United States)

    Skjaerbaek, C; Vahl, N; Frystyk, J; Hansen, T B; Jørgensen, J O; Hagen, C; Christiansen, J S; Orskov, H

    1997-08-01

    The objective of the present study was to compare fasting levels of free IGF-I in serum from patients with adult onset growth hormone deficiency (GHD) and from healthy volunteers, and to examine the effect of GH replacement therapy in GHD on serum free IGF-I. Free IGF-I was measured using separation of free IGF-I by ultrafiltration in serum samples from 42 healthy volunteers and 27 patients with GHD, in the latter before and after 1 year of treatment with GH (2 IU/m2) (n = 13) or placebo (n = 14). Free IGF-I was significantly decreased in patients with GHD (700 +/- 100 ng/l (mean +/- S.E.M.), range 55-2618 ng/l) compared with controls (1010 +/- 70 ng/l, range 231-2431 ng/l; P = 0.0016). Total IGF-I was 85 +/- 10 micrograms/l (GHD) and 160 +/- 10 micrograms/l (controls) (P < 0.0001). The ratio of free over total IGF-I was increased in GHD to 0.85 +/- 0.08% compared with 0.66 +/- 0.05% in controls (P = 0.04). In both GHD and controls, free IGF-I correlated significantly (P < 0.05) with total IGF-I (GHD r = 0.78; controls r = 0.42), IGFBP-1 (GHD r = -0.67; controls r = -0.46) and the molar ratio of total IGF-I over IGFBP-3 (GHD r = 0.58; controls r = 0.62). After 1 year of GH treatment, free IGF-I was increased to 2780 +/- 320 ng/l (P = 0.003) and total IGF-I was increased to 270 +/- 30 micrograms/l (P = 0.006) both of which values were greater than those in healthy volunteers. There were no changes in free or total IGF-I in the placebo-treated group. In conclusion, levels of free IGF-I are decreased in GHD, but measurements of free IGF-I in a single, fasting serum sample do not offer a better separation of patients with GHD from individuals with normal GH status than can be achieved by measurement of total IGF-I. One year of treatment with 2IU/m2 GH caused an increase of serum free IGF-I to supraphysiological levels.

  16. Elevated interferon gamma expression in the central nervous system of tumour necrosis factor receptor 1-deficient mice with experimental autoimmune encephalomyelitis

    DEFF Research Database (Denmark)

    Wheeler, Rachel D; Zehntner, Simone P; Kelly, Lisa M

    2006-01-01

    Inflammation in the central nervous system (CNS) can be studied in experimental autoimmune encephalomyelitis (EAE). The proinflammatory cytokines interferon-gamma (IFN-gamma) and tumour necrosis factor (TNF) are implicated in EAE pathogenesis. Signals through the type 1 TNF receptor (TNFR1) are r...

  17. Potential Contribution of Iron Deficiency and Multiple Factors to Anemia among 6- to 72-Month-Old Children in the Kokang Area of Myanmar

    OpenAIRE

    ZHAO, Ai; Gao, Hongchong; Li, Bo; Yu, Kai; Win, Naing Naing; Zhang, Yumei; Wang, Peiyu

    2015-01-01

    The prevalence of anemia among children in Myanmar has been reported to be among the highest in the world. This study was conducted to determine 1) the prevalence of anemia in preschool children and 2) risk factors associated with anemia. A total of 138 children aged from 6 to 72 months were recruited through cluster sampling from six villages in Kokang. Hemoglobin (Hb) concentration, blood trace elements, and anthropometric indicators were measured. Feces samples were collected to examine fo...

  18. Potential Contribution of Iron Deficiency and Multiple Factors to Anemia among 6- to 72-Month-Old Children in the Kokang Area of Myanmar

    OpenAIRE

    Zhao, Ai; Gao, Hongchong; Li, Bo; Yu, Kai; Win, Naing Naing; Zhang, Yumei; Wang, Peiyu

    2015-01-01

    The prevalence of anemia among children in Myanmar has been reported to be among the highest in the world. This study was conducted to determine 1) the prevalence of anemia in preschool children and 2) risk factors associated with anemia. A total of 138 children aged from 6 to 72 months were recruited through cluster sampling from six villages in Kokang. Hemoglobin (Hb) concentration, blood trace elements, and anthropometric indicators were measured. Feces samples were collected to examine fo...

  19. [Iodine deficiency in cardiovascular diseases].

    Science.gov (United States)

    Molnár, I; Magyari, M; Stief, L

    1998-08-30

    The thyroid hormone deficiency on cardiovascular function can be characterized with decreased myocardial contractility and increased peripheral vascular resistance as well as with the changes in lipid metabolism. 42 patients with cardiovascular disease (mean age 65 +/- 13 yr, 16 males) were investigated if iodine insufficiency can play a role as a risk factor for the cardiovascular diseases. The patients were divided in 5 subgroups on the ground of the presence of hypertension, congestive heart failure, cardiomyopathy, coronary disfunction and arrhythmia. Urine iodine concentration (5.29 +/- 4.52 micrograms/dl) was detected with Sandell-Kolthoff colorimetric reaction. The most decreased urine iodine concentration was detected in the subgroups with arrhythmia and congestive heart failure (4.7 +/- 4.94 micrograms/dl and 4.9 +/- 4.81 micrograms/dl, respectively). An elevated TSH level was found by 3 patients (5.3 +/- 1.4 mlU/l). An elevation in lipid metabolism (cholesterol, triglyceride) associated with all subgroups without arrhythmia. In conclusion, the occurrence of iodine deficiency in cardiovascular disease is frequent. Iodine supplementation might prevent the worsing effect of iodine deficiency on cardiovascular disease.

  20. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Events Spokespeople Email Alerts E-Newsletters About NHLBI Organization NHLBI Director Budget, Planning, & Legislative Advisory Committees Jobs ... severity of the condition. Treatments may include dietary changes, medicines, and surgery. Severe iron-deficiency anemia may ...