Sample records for developement aplastic crises
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Minhas, Parminder S; K Virdi, Jaspreet; Patel, Rajeshkumar
2017-07-01
Splenic dysfunction is a major feature of sickle cell disease (SCD) and can manifest as acute splenic sequestration crisis (ASSC), which is the earliest life-threatening complication seen in patients with SCD. Aplastic crisis is another potentially deadly complication of sickle cell disease that develops when erythrocyte production temporarily drops. Infection with parvovirus B-19 frequently causes aplastic crises. These two complications are known to be mutually exclusive due to their classic presentation signs and symptoms but there have been few cases where a patient can have concomitant presentation of both phenomena, which can result in a fatal outcome. These few cases force us to rethink the etiology and subsequent management guidelines of these complications. We present to you a case of an unfortunate 23-year-old female who had both complications occurring at the same time, resulting in death.
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Haddy, T B
1982-01-01
Erythropoietin responsible for the hormonal regulation of red blood cell production. Its formation is largely controlled by the kidneys. A number of assay methods for erythropoietin are available. Asymptomatic patients with sickle cell disease have elevated erythropoietin levels, as expected with chronic hemolysis. When complicated by chronic renal failure, erythropoietin levels do not rise appropriately. Chronic infection has not been studied, but the erythropoietin response in acute infection does not seem to conform to a pattern. Aplastic crises are characterized by very high levels of erythropoietin, suggesting bone marrow suppression, but events that trigger the crises remain obscure. In vaso-occlusive crises, there is also some suggestion of mild and transient lack of bone marrow response. Patients with sickle cell disease, with their chronic high erythropoietin anemia and susceptibility to altered states, are uniquely suited for investigating the physiology of erythropoietin, especially under the constraints of present assay methods.
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Severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia.
Scott, Angela; Glover, Jason; Skoda-Smith, Suzanne; Torgerson, Troy R; Xu, Min; Burroughs, Lauri M; Woolfrey, Ann E; Fleming, Mark D; Shimamura, Akiko
2015-11-01
Aplastic anemia in the neonate is rare. We report a case of severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia. This report highlights the importance of considering SCID early in the evaluation of neonatal aplastic anemia prior to the development of infectious complications. © 2015 Wiley Periodicals, Inc.
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A plasma microRNA signature as a biomarker for acquired aplastic anemia.
Hosokawa, Kohei; Kajigaya, Sachiko; Feng, Xingmin; Desierto, Marie J; Fernandez Ibanez, Maria Del Pilar; Rios, Olga; Weinstein, Barbara; Scheinberg, Phillip; Townsley, Danielle M; Young, Neal S
2017-01-01
Aplastic anemia is an acquired bone marrow failure characterized by marrow hypoplasia, a paucity of hematopoietic stem and progenitor cells, and pancytopenia of the peripheral blood, due to immune attack on the bone marrow. In aplastic anemia, a major challenge is to develop immune biomarkers to monitor the disease. We measured circulating microRNAs in plasma samples of aplastic anemia patients in order to identify disease-specific microRNAs. A total of 179 microRNAs were analyzed in 35 plasma samples from 13 aplastic anemia patients, 11 myelodysplastic syndrome patients, and 11 healthy controls using the Serum/Plasma Focus microRNA Polymerase Chain Reaction Panel. Subsequently, 19 microRNAs from the discovery set were investigated in the 108 plasma samples from 41 aplastic anemia patients, 24 myelodysplastic syndrome patients, and 43 healthy controls for validation, confirming that 3 microRNAs could be validated as dysregulated (>1.5-fold change) in aplastic anemia, compared to healthy controls. MiR-150-5p (induction of T-cell differentiation) and miR-146b-5p (involvement in the feedback regulation of innate immune response) were elevated in aplastic anemia plasma, whereas miR-1 was decreased in aplastic anemia. By receiver operating characteristic curve analysis, we developed a logistic model with these 3 microRNAs that enabled us to predict the probability of a diagnosis of aplastic anemia with an area under the curve of 0.86. Dysregulated expression levels of the microRNAs became normal after immunosuppressive therapy at 6 months. Specifically, miR-150-5p expression was significantly reduced after successful immunosuppressive therapy, but did not change in non-responders. We propose 3 novel plasma biomarkers in aplastic anemia, in which miR-150-5p, miR-146b-5p, and miR-1 can serve for diagnosis and miR-150-5p for disease monitoring. Clinicaltrials.gov identifiers:00260689, 00217594, 00961064. Copyright© Ferrata Storti Foundation.
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Hepatitis-associated aplastic anaemia: a poor prognosis.
Gonçalves, Vivian; Calado, Rita; Palaré, Maria João; Ferrão, Anabela; Morais, Anabela
2013-02-13
A 13-year-old boy presented with spontaneous skin and mucosal bleeds 3 weeks after acute hepatitis of unknown aetiology. Laboratory analyses revealed pancytopenia and bone marrow biopsy that confirmed the diagnosis of aplastic anaemia. Other causes of congenital and acquired aplastic anaemia were excluded. He was diagnosed with hepatitis-associated aplastic anaemia. He developed a critical clinical condition, becoming totally dependent on erythrocyte and platelet transfusions, and severe neutropenia, which led to invasive bacterial infection. He died due to sepsis with multiple organ failure 3 months after admission.
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Mohamed, M; Bates, G; Richardson, D; Burrows, L
2014-09-01
A 60-year-old woman was diagnosed with non-severe aplastic anaemia when she presented with anaemia and thrombocytopenia. She developed recurrent life-threatening hypotensive reactions during transfusion of leukodepleted platelet concentrates, and washed platelet concentrates prevented the development of such reactions subsequently. A paroxysmal nocturnal haemoglobinuria clone was detected on investigating for aplastic anaemia, which has been speculated to play a role in the recurrent hypotensive reactions. © 2014 The Authors; Internal Medicine Journal © 2014 Royal Australasian College of Physicians.
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Aplastic Anemia in Two Patients with Sex Chromosome Aneuploidies.
Rush, Eric T; Schaefer, G Bradley; Sanger, Warren G; Coccia, Peter F
2015-01-01
Sex chromosome aneuploidies range in incidence from rather common to exceedingly rare and have a variable phenotype. We report 2 patients with sex chromosome aneuploidies who developed severe aplastic anemia requiring treatment. The first patient had tetrasomy X (48,XXXX) and presented at 9 years of age, and the second patient had trisomy X (47,XXX) and presented at 5 years of age. Although aplastic anemia has been associated with other chromosomal abnormalities, sex chromosome abnormalities have not been traditionally considered a risk factor for this condition. A review of the literature reveals that at least one other patient with a sex chromosome aneuploidy (45,X) has suffered from aplastic anemia and that other autosomal chromosomal anomalies have been described. Despite the uncommon nature of each condition, it is possible that the apparent association is coincidental. A better understanding of the genetic causes of aplastic anemia remains important. © 2015 S. Karger AG, Basel.
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Kim, Sung-Yong; Le Rademacher, Jennifer; Antin, Joseph H; Anderlini, Paolo; Ayas, Mouhab; Battiwalla, Minoo; Carreras, Jeanette; Kurtzberg, Joanne; Nakamura, Ryotaro; Eapen, Mary; Deeg, H Joachim
2014-12-01
A proportion of patients with aplastic anemia who are treated with immunosuppressive therapy develop clonal hematologic disorders, including post-aplastic anemia myelodysplastic syndrome. Many will proceed to allogeneic hematopoietic stem cell transplantation. We identified 123 patients with post-aplastic anemia myelodysplastic syndrome who from 1991 through 2011 underwent allogeneic hematopoietic stem cell transplantation, and in a matched-pair analysis compared outcome to that in 393 patients with de novo myelodysplastic syndrome. There was no difference in overall survival. There were no significant differences with regard to 5-year probabilities of relapse, non-relapse mortality, relapse-free survival and overall survival; these were 14%, 40%, 46% and 49% for post-aplastic anemia myelodysplastic syndrome, and 20%, 33%, 47% and 49% for de novo myelodysplastic syndrome, respectively. In multivariate analysis, relapse (hazard ratio 0.71; P=0.18), non-relapse mortality (hazard ratio 1.28; P=0.18), relapse-free survival (hazard ratio 0.97; P=0.80) and overall survival (hazard ratio 1.02; P=0.88) of post-aplastic anemia myelodysplastic syndrome were similar to those of patients with de novo myelodysplastic syndrome. Cytogenetic risk was independently associated with overall survival in both groups. Thus, transplant success in patients with post-aplastic anemia myelodysplastic syndrome was similar to that in patients with de novo myelodysplastic syndrome, and cytogenetics was the only significant prognostic factor for post-aplastic anemia myelodysplastic syndrome patients. Copyright© Ferrata Storti Foundation.
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Penkert, Rhiannon R; Lavoie, Paul; Tang, Li; Sun, Yilun; Hurwitz, Julia L
2016-01-01
Parvovirus B19 infection causes transient aplastic crisis in sickle cell disease (SCD) due to a temporary interruption in the red blood cell production. Toxicity from hydroxyurea includes anemia and reticulocytopenia, both of which also occur during a transient aplastic crisis event. Hydroxyurea inhibits proliferation of hematopoietic cells and may be immunosuppressive. We postulated that hydroxyurea could exacerbate parvovirus B19-induced aplastic crisis and inhibit the development of specific immune responses in children with SCD. We conducted a retrospective review of parvovirus B19 infection in 330 children with SCD. Altogether there were 120 known cases of aplastic crisis attributed to parvovirus B19 infection, and 12% of children were on hydroxyurea treatment during the episode. We evaluated hematological and immune responses. Children with HbSS or HbSβ0-thalassemia treated with hydroxyurea, when compared with untreated children, required fewer transfusions and had higher Hb concentration nadir during transient aplastic crisis. Duration of hospital stays was no different between hydroxyurea-treated and untreated groups. Children tested within a week following aplastic crisis were positive for parvovirus-specific IgG. Immune responses lasted for the duration of the observation period, up to 13 years after transient aplastic crisis, and there were no repeat aplastic crisis episodes. The frequencies of parvovirus-specific antibodies in all children with SCD increased with age, as expected due to the increased likelihood of a parvovirus exposure, and were comparable to frequencies reported for healthy children. Approximately one-third of children had a positive parvovirus B19-specific IgG test without a documented history of transient aplastic crisis, and 64% of them were treated with hydroxyurea. Hydroxyurea may reduce requirements for blood transfusions and may attenuate symptoms during transient aplastic crisis episodes caused by parvovirus B19 infections. Children with SCD, whether treated or untreated with hydroxyurea, generate sustained and protective parvovirus B19-specific immune responses. PMID:26940953
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Hankins, Jane S; Penkert, Rhiannon R; Lavoie, Paul; Tang, Li; Sun, Yilun; Hurwitz, Julia L
2016-04-01
Parvovirus B19 infection causes transient aplastic crisis in sickle cell disease (SCD) due to a temporary interruption in the red blood cell production. Toxicity from hydroxyurea includes anemia and reticulocytopenia, both of which also occur during a transient aplastic crisis event. Hydroxyurea inhibits proliferation of hematopoietic cells and may be immunosuppressive. We postulated that hydroxyurea could exacerbate parvovirus B19-induced aplastic crisis and inhibit the development of specific immune responses in children with SCD. We conducted a retrospective review of parvovirus B19 infection in 330 children with SCD. Altogether there were 120 known cases of aplastic crisis attributed to parvovirus B19 infection, and 12% of children were on hydroxyurea treatment during the episode. We evaluated hematological and immune responses. Children with HbSS or HbSβ(0)-thalassemia treated with hydroxyurea, when compared with untreated children, required fewer transfusions and had higher Hb concentration nadir during transient aplastic crisis. Duration of hospital stays was no different between hydroxyurea-treated and untreated groups. Children tested within a week following aplastic crisis were positive for parvovirus-specific IgG. Immune responses lasted for the duration of the observation period, up to 13 years after transient aplastic crisis, and there were no repeat aplastic crisis episodes. The frequencies of parvovirus-specific antibodies in all children with SCD increased with age, as expected due to the increased likelihood of a parvovirus exposure, and were comparable to frequencies reported for healthy children. Approximately one-third of children had a positive parvovirus B19-specific IgG test without a documented history of transient aplastic crisis, and 64% of them were treated with hydroxyurea. Hydroxyurea may reduce requirements for blood transfusions and may attenuate symptoms during transient aplastic crisis episodes caused by parvovirus B19 infections. Children with SCD, whether treated or untreated with hydroxyurea, generate sustained and protective parvovirus B19-specific immune responses. © 2016 by the Society for Experimental Biology and Medicine.
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[Aplastic anaemia associated with pregnancy].
Bozhinova, S; Kirovakov, Zl; Porozhanova, K; Kostova, S; Bozhinov, P
2012-01-01
Aplastic anaemia is rear disease caused by destruction of pluripotent stem cells in bone marrow. Pregnancy is one of the main factor that lead to immunosuppression. During pregnancy aplastic anaemia could be life-threatening for both mother and child, because of the variety of complications like bleeding and infections. We introduce the first case of pregnant woman with aplastic anaemia in Bulgaria. The woman was diagnosed in 12-13 gestational week. All biometric characteristics of the foetus were normal. The patient was consulted with oncohaematologists, pediatricians, specialists of Obstetrics and Gynaecology, and intensivists. Methylprednisolone, antibiotics, packed cells and platelet transfusions were initiated. However, the moment for interruption of the pregnancy was missed (first trimester). The woman developed a fever and vomited bloody material. Despite the optimal supportive treatment, the patient died. The pathoanatomy diagnose is Aplastic anaemia, induced by the pregnancy. From our experience with that case and other references from the literature we conclude that all pregnant woman with aplastic anaemia should interrupt their pregnancy during first trimester. In those patients who are diagnosed at later terms of pregnancy very supportive infusions and immunosuppressive therapy should be made, including antithymocyte globulin and/or cyclosporine. Women with no improvement from that therapy should achieve a bone-marrow transplantation.
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So, K; Macquillan, G; Garas, G; Delriviere, L; Mitchell, A; Speers, D; Mews, C; Augustson, B; de Boer, W B; Baker, D; Jeffrey, G P
2007-03-01
An 11-year-old boy presented with hepatic failure secondary to parvovirus B19 infection, requiring urgent liver transplantation. His recovery was complicated by primary Epstein-Barr virus and cytomegalovirus infections. He subsequently developed aplastic anaemia that has been refractory to antithymocyte globulin and cyclosporine therapy and may now require bone marrow transplantation. We present this case to emphasize parvovirus as a rare cause of hepatic failure and of aplastic anaemia as a complication of the virus.
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Diagnostic and management dilemma of a pancreas-kidney transplant recipient with aplastic anaemia.
Viecelli, Andrea; Hessamodini, Hannah; Augustson, Bradley; Lim, Wai Hon
2014-09-25
We report a case of a 57-year-old woman with type I diabetes who had received a simultaneous pancreas-kidney (SPK) transplant maintained on tacrolimus, mycophenolic acid (MPA) and prednisolone. Her renal allograft failed 6 years post-transplant but she continued to have a normal functioning pancreatic allograft. Over the course of 5 years, she developed progressive bone marrow failure with repeat bone marrow aspirates demonstrating an evolution from erythroid hypoplasia to hypocellular marrow and eventual aplastic anaemia despite discontinuation of MPA and reduction of tacrolimus. She was transfusion-dependent and had frequent admissions for sepsis. Despite treatment with antithymocyte globulin and cyclosporine for aplastic anaemia, she developed fatal invasive pulmonary aspergillosis within 3 weeks of treatment. Even though the cause of aplastic anaemia is likely multifactorial, this case highlights the difficulty in balancing the need for versus the risk of ongoing immunosuppression in a SPK transplant recipient who continues to have normal pancreatic graft function. 2014 BMJ Publishing Group Ltd.
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Special Issues for People with Aplastic Anemia
... Menu Donate Special Issues for People with Aplastic Anemia Because you have aplastic anemia , everyday events can ... bleeding, such as contact sports. Pregnancy and Aplastic Anemia Pregnancy is possible for women who have been ...
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Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.
Zikidou, Panagiota; Grapsa, Anastassia; Bezirgiannidou, Zoe; Chatzimichael, Athanassios; Mantadakis, Elpis
2018-01-01
Human parvovirus B19 (HPV-B19) is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals. We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thrombocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with the presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection. The patient was initially managed with corticosteroids, but both cytopenias resolved only after administration of intravenous gamma globulin 0.8g/kg. Acute parvovirus B19 infection should be suspected in patients with immunologic diseases, who present reticulocytopenic hemolytic anemia and thrombocytopenia. In this setting, intravenous gamma globulin is effective for both cytopenias.
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Bär, Christian; Povedano, Juan Manuel; Serrano, Rosa; Benitez-Buelga, Carlos; Popkes, Miriam; Formentini, Ivan; Bobadilla, Maria; Bosch, Fatima; Blasco, Maria A
2016-04-07
Aplastic anemia is a fatal bone marrow disorder characterized by peripheral pancytopenia and marrow hypoplasia. The disease can be hereditary or acquired and develops at any stage of life. A subgroup of the inherited form is caused by replicative impairment of hematopoietic stem and progenitor cells due to very short telomeres as a result of mutations in telomerase and other telomere components. Abnormal telomere shortening is also described in cases of acquired aplastic anemia, most likely secondary to increased turnover of bone marrow stem and progenitor cells. Here, we test the therapeutic efficacy of telomerase activation by using adeno-associated virus (AAV)9 gene therapy vectors carrying the telomerase Tert gene in 2 independent mouse models of aplastic anemia due to short telomeres (Trf1- and Tert-deficient mice). We find that a high dose of AAV9-Tert targets the bone marrow compartment, including hematopoietic stem cells. AAV9-Tert treatment after telomere attrition in bone marrow cells rescues aplastic anemia and mouse survival compared with mice treated with the empty vector. Improved survival is associated with a significant increase in telomere length in peripheral blood and bone marrow cells, as well as improved blood counts. These findings indicate that telomerase gene therapy represents a novel therapeutic strategy to treat aplastic anemia provoked or associated with short telomeres. © 2016 by The American Society of Hematology.
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Hama, Asahito; Takahashi, Yoshiyuki; Muramatsu, Hideki; Ito, Masafumi; Narita, Atsushi; Kosaka, Yoshiyuki; Tsuchida, Masahiro; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji
2015-11-01
The 2008 World Health Organization classification proposed a new entity in childhood myelodysplastic syndrome, refractory cytopenia of childhood. However, it is unclear whether this morphological classification reflects clinical outcomes. We retrospectively reviewed bone marrow morphology in 186 children (median age 8 years; range 1-16 years) who were enrolled in the prospective study and received horse antithymocyte globulin and cyclosporine between July 1999 and November 2008. The median follow-up period was 87 months (range 1-146 months). Out of 186 patients, 62 (33%) were classified with aplastic anemia, 94 (49%) with refractory cytopenia of childhood, and 34 (18%) with refractory cytopenia with multilineage dysplasia. Aplastic anemia patients received granulocyte colony-stimulating factor more frequently and for longer durations than other patients (P<0.01). After six months, response rates to immunosuppressive therapy were not significantly different among the 3 groups. Acquisition of chromosomal abnormalities was observed in 5 patients with aplastic anemia, 4 patients with refractory cytopenia of childhood, and 3 patients with refractory cytopenia with multilineage dysplasia. Although the cumulative incidence of total clonal evolution at ten years was not significantly different among the 3 groups, the cumulative incidence of monosomy 7 development was significantly higher in aplastic anemia than in the other groups (P=0.02). Multivariate analysis revealed that only granulocyte colony-stimulating factor administration duration of 40 days or more was a significant risk factor for monosomy 7 development (P=0.02). These findings suggest that even the introduction of a strict morphological distinction from hypoplastic myelodysplastic syndrome cannot eradicate clonal evolution in children with aplastic anemia. Copyright© Ferrata Storti Foundation.
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Lengline, Etienne; Drenou, Bernard; Peterlin, Pierre; Tournilhac, Olivier; Abraham, Julie; Berceanu, Ana; Dupriez, Brigitte; Guillerm, Gaelle; Raffoux, Emmanuel; de Fontbrune, Flore Sicre; Ades, Lionel; Balsat, Marie; Chaoui, Driss; Coppo, Paul; Corm, Selim; Leblanc, Thierry; Maillard, Natacha; Terriou, Louis; Socié, Gerard; de Latour, Regis Peffault
2018-02-01
Few therapeutic options are available for patients with aplastic anemia who are ineligible for transplantation or refractory to immunosuppressive therapy. Eltrombopag was recently shown to produce trilineage responses in refractory patients. However, the effects of real-life use of this drug remain unknown. This retrospective study (2012-2016) was conducted by the French Reference Center for Aplastic Anemia on patients with relapsed/refractory aplastic anemia, and patients ineligible for antithymocyte globulin or transplantation, who received eltrombopag for at least 2 months. Forty-six patients with aplastic anemia were given eltrombopag without prior antithymocyte globulin treatment (n=11) or after antithymocyte globulin administration (n=35) in a relapsed/refractory setting. Eltrombopag (median daily dose 150 mg) was introduced 17 months (range, 8-50) after the diagnosis of aplastic anemia. At last followup, 49% were still receiving treatment, 9% had stopped due to a robust response, 2% due to toxicity and 40% due to eltrombopag failure. Before eltrombopag treatment, all patients received regular transfusions. The overall rates of red blood cell and platelet transfusion independence were 7%, 33%, 46% and 46% at 1, 3, 6 months and last follow-up. Responses were slower to develop in antithymocyte treatment-naïve patients. In patients achieving transfusion independence, hemoglobin concentration and platelet counts improved by 3 g/dL (interquartile range, 1.4-4.5) and 42×10 9 /L (interquartile range, 11-100), respectively. Response in at least one lineage (according to National Institutes of Health criteria) was observed in 64% of antithymocyte treatment-naïve and 74% of relapsed/refractory patients, while trilineage improvement was observed in 27% and 34%, respectively. We found high rates of hematologic improvement and transfusion independence in refractory aplastic anemia patients but also in patients ineligible for antithymocyte globulin receiving first-line treatment. In conclusion, elderly patients unfit for antithymocyte globulin therapy may benefit from eltrombopag. Copyright© 2018 Ferrata Storti Foundation.
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Chatterjee, Ritam; Law, Sujata
2018-01-01
Aplastic anemia or bone marrow failure often develops as an effect of chemotherapeutic drug application for the treatment of various pathophysiological conditions including cancer. The long-term bone marrow injury affects the basic hematopoietic population including hematopoietic stem/progenitor cells (HSPCs). The present study aimed in unearthing the underlying mechanisms of chemotherapeutics mediated bone marrow aplasia with special focus on altered redox status and associated effects on hematopoietic microenvironment and epigenetic status of hematopoietic cells. The study involves the development of busulfan and cyclophosphamide mediated mouse model for aplastic anemia, characterization of the disease with blood and marrow analysis, cytochemical examinations of bone marrow, flowcytometric analysis of hematopoietic population and microenvironmental components, determination of ROS generation, apoptosis profiling, expressional studies of Notch-1 signaling cascade molecules, investigation of epigenetic modifications including global CpG methylation of DNA, phosphorylation of histone-3 with their effects on bone marrow kinetics and expressional analysis of the anti-oxidative molecules viz; SOD-2 and Sdf-1. Severe hematopoietic catastrophic condition was observed during aplastic anemia which involved peripheral blood pancytopenia, marrow hypocellularity and decreased hematopoietic stem/progenitor population. Generation of ROS was found to play a central role in the cellular devastation in aplastic marrow which on one hand can be correlated with the destruction of hematopoiesis supportive niche components and alteration of vital Notch-1 signaling and on other hand was found to be associated with the epigenetic chromatin modifications viz; global DNA CpG hypo-methylation, histone-3 phosphorylation promoting cellular apoptosis. Decline of anti-oxidant components viz; Sdf-1 and SOD-2 hinted towards the irreversible nature of the oxidative damage during marrow aplasia. Collectively, the findings hinted towards the mechanistic correlation among ROS generation, microenvironmental impairment and epigenetic alterations that led to hematopoietic catastrophe under aplastic stress. The findings may potentiate successful therapeutic strategy development for the dreadful condition concerned. Copyright © 2017 Elsevier GmbH. All rights reserved.
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Maluf, Eliane; Hamerschlak, Nelson; Cavalcanti, Alexandre Biasi; Júnior, Álvaro Avezum; Eluf-Neto, José; Falcão, Roberto Passetto; Lorand-Metze, Irene G.; Goldenberg, Daniel; Santana, Cézar Leite; de Oliveira Werneck Rodrigues, Daniela; da Motta Passos, Leny Nascimento; Rosenfeld, Luis Gastão Mange; Pitta, Marimilia; Loggetto, Sandra; Feitosa Ribeiro, Andreza A.; Velloso, Elvira Deolinda; Kondo, Andrea Tiemi; de Miranda Coelho, Erika Oliveira; Pintão, Maria Carolina Tostes; de Souza, Hélio Moraes; Borbolla, José Rafael; Pasquini, Ricardo
2009-01-01
Background Associations between aplastic anemia and numerous drugs, pesticides and chemicals have been reported. However, at least 50% of the etiology of aplastic anemia remains unexplained. Design and Methods This was a case-control, multicenter, multinational study, designed to identify risk factors for agranulocytosis and aplastic anemia. The cases were patients with diagnosis of aplastic anemia confirmed through biopsy or bone marrow aspiration, selected through an active search of clinical laboratories, hematology clinics and medical records. The controls did not have either aplastic anemia or chronic diseases. A total of 224 patients with aplastic anemia were included in the study, each case was paired with four controls, according to sex, age group, and hospital where the case was first seen. Information was collected on demographic data, medical history, laboratory tests, medications, and other potential risk factors prior to diagnosis. Results The incidence of aplastic anemia was 1.6 cases per million per year. Higher rates of benzene exposure (≥30 exposures per year) were associated with a greater risk of aplastic anemia (odds ratio, OR: 4.2; 95% confidence interval, CI: 1.82–9.82). Individuals exposed to chloramphenicol in the previous year had an adjusted OR for aplastic anemia of 8.7 (CI: 0.87–87.93) and those exposed to azithromycin had an adjusted OR of 11.02 (CI 1.14–108.02). Conclusions The incidence of aplastic anemia in Latin America countries is low. Although the research study centers had a high coverage of health services, the underreporting of cases of aplastic anemia in selected regions can be discussed. Frequent exposure to benzene-based products increases the risk for aplastic anemia. Few associations with specific drugs were found, and it is likely that some of these were due to chance alone. PMID:19734415
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Aksoy, M.
The hematotoxicity of benzene exposure has been well known for a century. Benzene causes leukocytopenia, thrombocytopenia, pancytopenia, etc. The clinical and hematologic picture of aplastic anemia resulting from benzene exposure is not different from classical aplastic anemia; in some cases, mild bilirubinemia, changes in osmotic fragility, increase in lactic dehydrogenase and fecal urobilinogen, and occasionally some neurological abnormalities are found. Electromicroscopic findings in some cases of aplastic anemia with benzene exposure were similar to those observed by light microscopy. Benzene hepatitis-aplastic anemia syndrome was observed in a technician with benzene exposure. Ten months after occurrence of hepatitis B, a severemore » aplastic anemia developed. The first epidemiologic study proving the leukemogenicity of benzene was performed between 1967 and 1973 to 1974 among shoe workers in Istanbul. The incidence of leukemia was 13.59 per 100,000, which is a significant increase over that of leukemia in the general population. Following the prohibition and discontinuation of the use of benzene in Istanbul, there was a striking decrease in the number of leukemic shoe workers in Istanbul. In 23.7% of the series, consisting of 59 leukemic patients with benzene exposure, there was a preceding pancytopenic period. Furthermore, a familial connection was found in 10.2% of them. The 89.8% of the series showed the findings of acute leukemia. The possible factors that may determine the types of leukemia in benzene toxicity are discussed. The possible role of benzene exposure is presented in the development of malignant lymphoma, multiple myeloma, and lung cancer.« less
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Pérard, Baptiste; Rougeron, Amandine; Favre, Simon; Accoceberry, Isabelle; Vigouroux, Stéphane; Mohr, Catherine; Milpied, Noël
2015-01-01
We report a case of a 51-year old man with a severe aplastic anemia who developed an invasive trichosporonosis to Trichosporon faecale with fungemia and skin lesions during severe neutropenia. The treatment was successful before neutrophil recovery with a combination of voriconazole and liposomal amphotericin B. PMID:26199866
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Pérard, Baptiste; Rougeron, Amandine; Favre, Simon; Accoceberry, Isabelle; Vigouroux, Stéphane; Mohr, Catherine; Milpied, Noël
2015-09-01
We report a case of a 51-year old man with a severe aplastic anemia who developed an invasive trichosporonosis to Trichosporon faecale with fungemia and skin lesions during severe neutropenia. The treatment was successful before neutrophil recovery with a combination of voriconazole and liposomal amphotericin B.
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Immunosuppressive therapy for transplant-ineligible aplastic anemia patients.
Schrezenmeier, Hubert; Körper, Sixten; Höchsmann, Britta
2015-02-01
Aplastic anemia is a rare life-threatening bone marrow failure that is characterized by bicytopenia or pancytopenia in the peripheral blood and a hypoplastic or aplastic bone marrow. The patients are at risk of infection and hemorrhage due to neutropenia and thrombocytopenia and suffer from symptoms of anemia. The main treatment approaches are allogeneic stem cell transplantation and immunosuppression. Here, we review current standard immunosuppression and the attempts that have been made in the past two decades to improve results: review of recent developments also reveals that sometimes not only the advent of new drugs, good ideas and well-designed clinical trials decide the progress in the field but also marketing considerations of pharmaceutical companies. Aplastic anemia experts unfortunately had to face the situation that efficient drugs were withdrawn simply for marketing considerations. We will discuss the current options and challenges in first-line treatment and management of relapsing and refractory patients with an emphasis on adult patients. Some promising new approaches are currently under investigation in prospective, randomized trials.
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Mandal, Prakas Kumar; Baul, Suvraneel; Dolai, Tuphan Kanti; De, Rajib; Chakrabarti, Prantar
2017-03-01
Immune suppression is a crucial pillar for treatment of aplastic anemia. Cyclosporine monotherapy is an easily available, affordable therapeutic option with good safety profile. This prospective study was conducted over a period of 2 years from June 2012 to July 2014. The diagnosis and response to treatment of aplastic anemia was established as per published criteria. Follow up was done at 3 and 6 months in order to assess the response. 57 patients of acquired aplastic anemia with median age of 37 years (6 to 81 years) were included in the study. 35 (62 %) cases were severe aplastic anemai, 16 (28 %) non severe aplastic anemia and 6 (10 %) were very severe aplastic anemia. At 3 months overall response rate (OR) was 7 (14 %) and at 6 months the OR rate of 11 (19.6 %) was achieved. Transiently raised creatinine, liver function abnormality and gum hypertrophy were the main side effects observed in this cohort. Oral cyclosporine monotherapy at dose of 5 mg/kg/day is a relatively safe treatment option for resource poor patients with aplastic anemia.
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Hemoperitoneum from corpus luteum rupture in patients with aplastic anemia.
Wang, Huaquan; Guo, Lifang; Shao, Zonghong
2015-01-01
Aplastic anemia is a rare hematopoietic stem-cell disorder that results in pancytopenia and hypocellular bone marrow. Women with aplastic anemia usually are at increased risk of corpus luteum rupture due to thrombocytopenia and infection. Here we report two cases had hemoperitoneum from corpus luteum rupture in patients with aplastic anemia in our center. Case 1 involved two episodes of hemoperitoneum resulting from rupture of the corpus luteum in a 23-year-old unmarried female with severe aplastic anemia. This patient was managed conservatively with platelet and packed red cell transfusion. Case 2 involved two episodes of hemoperitoneum resulting from rupture of the corpus luteum in a 33-year-old married patient with aplastic anemia. Emergency laparoscopy revealed massive hemoperitoneum. Bilateral salpingo-oophorectomy were performed successively with platelet and packed red cell transfusion. Hemoperitoneum resulting from a ruptured corpus luteum is a life-threatening condition in patients with aplastic anemia. Prompt and appropriate evaluation of corpus luteum rupture and emergent therapy are needed.
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Chatterjee, Ritam; Chattopadhyay, Sukalpa; Law, Sujata
2016-11-01
Aplastic anemia, the paradigm of bone marrow failure, is characterized by pancytopenic peripheral blood and hypoplastic bone marrow. Among various etiologies, inappropriate use of DNA alkylating drugs like cyclophosphamide and busulfan often causes the manifestation of the dreadful disease. Cell cycle impairment in marrow hematopoietic stem/progenitor compartment together with cellular apoptosis has been recognized as culpable factors behind aplastic pathophysiologies. However, the intricate molecular mechanisms remain unrevealed till date. In the present study, we have dealt with the mechanistic intervention of the disease by peripheral blood hemogram, bone marrow histopathology, cytopathology, hematopoietic kinetic study, scanning electron microscopy, DNA damage assessment and flowcytometric analysis of cellular proliferation and apoptosis in hematopoietic stem/progenitor cell (HSPC) rich marrow compartment using busulfan and cyclophosphamidemediated mouse model. To unveil the molecular mechanisms behind aplastic pathophysiology, we further investigated the role of some crucial mitotic and apoptotic regulators like Protein kinase-B (PKB), Gsk-3β, Cyclin-D1, PP2A, Cdc25c, Plk-1, Aurora kinase-A, Chk-1 regarding the hematopoietic catastrophe. Our observations revealed that the alteration of PKB-GSK-3β axis, Plk-1, and Aurora kinase-A expressions in HSPC compartment due to DNA damage response was associated with the proliferative impairment and apoptosis during aplastic anemia. The study established the correlation between the accumulation of DNA damage and alteration of the mentioned molecules in aplastic HSPCs that lead to the hematopoietic catastrophe. We anticipate that our findings will be beneficial for developing better therapeutic strategies for the dreadful disease concerned.
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Sun, Yuan-Xin; Li, Hui; Feng, Qi; Li, Xin; Yu, Ying-Yi; Zhou, Li-Wei; Gao, Yan; Li, Guo-Sheng; Ren, Juan; Ma, Chun-Hong; Gao, Cheng-Jiang; Peng, Jun
2017-01-24
Acquired aplastic anemia is an idiopathic paradigm of human bone marrow failure syndrome, which involves active destruction of hematopoietic stem cells and progenitors by cytotoxic T cells in the bone marrow. Aberrant expression of microRNAs in T cells has been shown to lead to development of certain autoimmune diseases. In the present study, we performed a microarray analysis of miRNA expression in bone marrow CD3+ T cells from patients with aplastic anemia and healthy controls. Overexpression of miR34a and underexpression of its target gene diacylglycerol kinase (DGK) ζ in bone marrow mononuclear cells were validated in 41 patients and associated with the severity of aplastic anemia. Further, the level of miR34a was higher in naïve T cells from patients than from controls. The role of miR34a and DGKζ in aplastic anemia was investigated in a murine model of immune-mediated bone marrow failure using miR34a-/- mice. After T-cell receptor stimulation in vitro, lymph node T cells from miR34a-/- mice demonstrated reduced activation and proliferation accompanied with a less profound down-regulation of DGKζ expression and decreased ERK phosphorylation compared to those from wild-type C57BL6 control mice. Infusion of 5 × 106 miR34a-/- lymph node T cells into sublethally irradiated CB6F1 recipients led to increased Lin-Sca1+CD117+ cells and less vigorous expansion of CD8+ T cells than injection of same number of wild-type lymph node cells. Our study demonstrates that the miR34a/DGKζ dysregulation enhances T-cell activation in aplastic anemia and targeting miR34a may represent a novel molecular therapeutic approach for patients with aplastic anemia.
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[Meta-analysis of association between organophosphorus pesticides and aplastic anemia].
Zhang, Ji; Yang, Tubao
2015-09-01
To evaluate the association between organophosphorus pesticides and aplastic anemia, and provide scientific evidence for the primary prevention of aplastic anemia. The published papers of case control studies on the association between organophosphorus pesticides and aplastic anemia from January 1990 to August 2014 were collected from Chinese BioMedical Literature Base (CBM), Chinese National Knowledge Infrastructure (CNKI), PubMed and EMBASE. The papers which met the inclusion criteria were evaluated. The pooled odds ratios (OR) and 95% confidence interval (CI) of organophosphorus pesticides were calculated with software Review Manager 5.0. Subgroup analysis were conducted for different population and different usage of organophosphorus pesticides. A total of 9 papers were selected, involving 5 833 subjects (1 404 cases and 4 429 controls). The results showed that organophosphorus pesticides could increase the risk of aplastic anemia (OR=1.97, 95% CI: 1.60-2.44) . Subgroup analysis showed that Asian (OR=2.01, 95% CI: 1.52-2.66) had higher risk of aplastic anemia than American or European (OR=1.93, 95% CI: 1.39-2.67) . Using pure organophosphorus pesticides (OR=2.15, 95% CI: 1.60-2.88) was more prone to cause aplastic anemia than using the mixture of organophosphorus pesticides (OR=1.82, 95% CI: 1.34-2.47). The analysis indicated that organophosphorus pesticides might be a risk factor for aplastic anemia. Reducing organophosphorus pesticides exposure in daily life and industrial or agricultural production could prevent the incidence of aplastic anemia.
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Osteonecrosis of the hip in patients with aplastic anemia.
Park, Jeongmi; Jun, Jeongsu; Kim, Yongsik; Lee, Jongwook; Kim, Chunchu; Hahn, Seongtae
2002-01-01
The incidence and clinical and magnetic resonance imaging features of osteonecrosis of the hip were evaluated in patients with aplastic anemia. Two hundred and forty-one patients with aplastic anemia were examined using MR imaging of bone marrow during the five years from 1994 to 1998. Osteonecrosis of the hip was observed on MR imaging in nineteen (15 males and 4 females, mean age 35 yr) of the 241 patients. It was present in both hips in 14 patients, and there were five cases with unilateral occurrence, with a total of 33 involved hips. All except for five hips with associated bone marrow edema revealed increased fatty marrow conversion in the proximal femoral metaphysis. In nine patients, osteonecrosis was detected without any pain. Five patients already had osteonecrosis before any medication was administered. Twelve patients received antilymphocyte globulin, and seven patients received a low dose of steroids before the MR diagnosis of osteonecrosis. Osteonecrosis of the hip frequently develops in patients with aplastic anemia (7.9%), associated with fatty marrow conversion of the proximal femoral metaphysis. PMID:12483006
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The cutting edge of aplastic anemia treatment.
Obara, Naoshi
2017-01-01
Aplastic anemia is a syndrome in which hematopoietic stem cells are decreased and bone marrow hypoplasia and pancytopenia are observed; it is considered as a T cell-mediated autoimmune disease. Recently, it has been reported that gene mutations suggestive of clonal hematopoiesis are detected in approximately one third of the patients with aplastic anemia. Among treatment approaches other than hematopoietic stem cell transplantation, immunosuppressive therapy with antithymocyte globulin (ATG) plus cyclosporin is a basic approach, although it has been shown that eltrombopag, a thrombopoietin receptor agonist, is effective and that the recovery of hematopoiesis in three blood lineage is observed in some patients. Studies on the optimum dose of ATG are in progress. Regarding hematopoietic stem cell transplantation for aplastic anemia, regimens are being designed in which cyclophosphamide as a pretreatment is reduced and fludarabine is instead used in combination for the reduction of cardiotoxicity. Because HLA haploidentical transplantation has been developed and its reports are increasing for patients who cannot find appropriate donors, transplantation may be possible in patients who had previously given up on it.
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Epidemiology of acquired aplastic anaemia in Pakistan.
Ehsan, Ayesha; Shah, Shahida Amjad Riaz; Ibrahim, Tayyaba
2011-01-01
Acquired aplastic anaemia is a rare disease which results in morbidity and mortality at a young age. This study was carried out to determine the clinical presentation, haematological parameters and association factors of acquired aplastic anaemia in a cohort of Pakistani patients. This was a cross-sectional study conducted at Haematology Department, Shaikh Zayed Hospital, Lahore over 7 years from June 2000 to July 2007. Eighty-two patients of acquired aplastic anaemia were enrolled in the study by non-probability purposive sampling. Their diagnosis was confirmed by complete blood count, bone marrow aspirate and trephine biopsy. The cohort was classified on the basis of severity and the epidemiological, clinical and haematological parameters were analysed. Of the 82 enrolled patients of acquired aplastic anaemia, 49 (59.8%) were males and 33 (40.2%) were females. Mean age of the patients was 27.93 +/- 18.7 years with a range of 1-80 years. The male to female ratio was 1.48:1. Bone marrow cellularity was less than 25 % in 31 (38.0%) cases and between 25-30% in 51 (62%) of patients. Most of the cases were clinically severe aplastic anaemia (68%). In 62 (76%) of the cases no association factors predisposing to aplastic anaemia could be identified. Acquired Aplastic anaemia is a disease of all ages. In the second decade and the elderly predominantly severe clinical stages were seen. Males presented at a younger age while females presented at all ages with a somewhat similar incidence. No association factors of Aplastic Anaemia could be identified in majority of the patients.
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Cytogenetic profile of aplastic anaemia in Indian children.
Gupta, Vineeta; Kumar, Akash; Saini, Isha; Saxena, Ajit Kumar
2013-03-01
Aplastic anaemia is a rare haematological disorder characterized by pancytopenia with a hypocellular bone marrow. It may be inherited/genetic or acquired. Acquired aplastic anaemia has been linked to many drugs, chemicals and viruses. Cytogenetic abnormalities have been reported infrequently with acquired aplastic anaemia. Majority of the studies are in adult patients from the West. We report here cytogenetic studies on paediatric patients with acquired aplastic anaemia seen in a tertiary care hospital in north India. Patients (n=71, age 4-14 yr) were diagnosed according to the guidelines of International Agranulocytosis and Aplastic Anaemia Study. Conventional cytogenetics with Giemsa Trypsin Giemsa (GTG) banding was performed. Karyotyping was done according to the International System for Human Cytogenetics Nomenclature (ISCN). Of the 71 patients, 42 had successful karyotyping where median age was 9 yr; of these 42, 27 (64.3%) patients had severe, nine (21.4%) had very severe and six (14.3%) had non severe aplastic anaemia. Five patients had karyotypic abnormalities with trisomy 12 (1), trisomy 8 (1) and monosomy 7 (1). Two patients had non numerical abnormalities with del 7 q - and t (5:12) in one each. Twenty nine patients had uninformative results. There was no difference in the clinical and haematological profile of patients with normal versus abnormal cytogenetics although the number of patients was small in the two groups. Five (11.9%) patients with acquired aplastic anaemia had chromosomal abnormalities. Trisomy was found to be the commonest abnormality. Cytogenetic abnormalities may be significant in acquired aplastic anaemia although further studies on a large sample are required to confirm the findings.
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Identification of novel microRNA signatures linked to acquired aplastic anemia.
Hosokawa, Kohei; Muranski, Pawel; Feng, Xingmin; Keyvanfar, Keyvan; Townsley, Danielle M; Dumitriu, Bogdan; Chen, Jichun; Kajigaya, Sachiko; Taylor, James G; Hourigan, Christopher S; Barrett, A John; Young, Neal S
2015-12-01
Emerging evidence indicates that microRNA control and modulate immunity. MicroRNA have not been investigated in acquired aplastic anemia, a T-cell-mediated immune disease. Analysis of 84 microRNA expression levels in CD4(+) and CD8(+) T cells of patients with aplastic anemia revealed concurrent down-regulation of miR-126-3p, miR-145-5p, miR-223-3p, and miR-199a-5p (>3-fold change, P<0.05) in both T-cell populations, which were unique in aplastic anemia compared to other hematologic disorders. MiR-126-3p and miR-223-3p were down-regulated in CD4(+) T effector memory cells, and miR-126-3p, miR-145-5p, and miR-223-3p were down-regulated in CD8(+) T effector memory and terminal effector cells. Successful immunosuppressive therapy was associated with restoration to normal expression levels of miR-126-3p, miR-145-5p, and miR-223-3p (>2-fold change, P<0.05). In CD4(+) and CD8(+) T cells in aplastic anemia patients, MYC and PIK3R2 were up-regulated and proved to be targets of miR-145-5p and miR-126-3p, respectively. MiR-126-3p and miR-145-5p knockdown promoted proliferation and increased interferon-γ and granzyme B production in both CD4(+) and CD8(+) T cells. Our work describes previously unknown regulatory roles of microRNA in T-cell activation in aplastic anemia, which may open a new perspective for development of effective therapy. Clinicaltrials.gov identifier: NCT 01623167. Copyright© Ferrata Storti Foundation.
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Meta-analysis of Huangqi injection for the adjunctive therapy of aplastic anemia
Zhu, Changtai; Gao, Yulu; Jiang, Ting; Hao, Cao; Gao, Zongshuai; Sun, Yongning
2015-01-01
Aplastic anemia therapy remains difficult, due to lack of effective treatment regimens. In recent years, Huangqi injection for the adjunctive therapy of aplastic anemia has been reported in many clinical trials. Considering that Huangqi injection may be a novel approach to aplastic anemia treatment, we conducted a meta-analysis of clinical controlled trials to assess the clinical value of Huangqi injection in the treatment of aplastic anemia. We searched the Chinese Biomedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), Chinese Scientific Journals Full-text Database (VIP), Wanfang Database, PubMed and EMBASE database to collect the data about the trials of Huangqi injection combined with androgens for treating aplastic anemia. A total of ten studies involving 720 patients with aplastic anemia were included in this study. The meta-analysis showed significant increases in the pool effectiveness rate, white blood cells (WBC), haematoglobin (Hb), platelets (PLT), and reticulocytes (Ret) between the experimental group versus the control group. No severe side effects were found in this study. However, the lower Jadad scores and asymmetric funnel plot degrades the validity of the meta-analysis as the clinical evidence. Therefore, Huangqi injection may significantly enhance the efficacy of androgens for aplastic anemia, suggesting that the novel approach of Chinese traditional medicine combined with Western medicine is promising. The exact outcome required confirmation with rigorously well-designed multi-center trials. PMID:26379817
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Sachdeva, Man Updesh Singh; Varma, Neelam; Chandra, Dinesh; Bose, Parveen; Malhotra, Pankaj; Varma, Subhash
2015-05-01
Flow cytometry is the gold standard methodology for screening of paroxysmal nocturnal hemoglobinuria. In the last few years, proaerolysin conjugated with fluorescein (FLAER) has become an important component of antibody panel used for the detection of paroxysmal nocturnal hemoglobinuria (PNH) clone. This study aimed to compare PNH clone detection by flow cytometry in the pre-FLAER era versus the FLAER era. This was a retrospective analysis of 4 years and included 1004 individuals screened for PNH clone, either presenting as hemolytic anemia or as aplastic anemia. In the pre-FLAER time period, the RBCs and neutrophils were screened with antibodies against CD55 and CD59. With the introduction of FLAER, neutrophils were screened with FLAER/CD24/CD15 and monocytes with FLAER/CD14/CD33 combination. A comparative analysis was done for detection of PNH clone in aplastic anemia patients versus non-aplastic anemia patients, as well as between pre-FLAER and FLAER era. Out of a total of 1004 individuals, 59 (5.8%) were detected to have PNH clone positivity. The frequency of PNH clone detected in aplastic anemia and non-aplastic anemia groups was 12.02 and 3.36%, respectively. The detection rate of PNH clone increased from 4.5% (32/711) in the pre-FLAER era to 9.2% (27/293) with the introduction of FLAER. However, this increase could be attributed to increased detection of PNH clone in the aplastic anemia group, which showed a significant increase from 8.3 to 18.2% after use of FLAER. In the non-aplastic group, PNH clone was detected with similar frequencies before and after use of FLAER (3.2 versus 3.8%, respectively). Mean PNH clone size was lower in the aplastic anemia group when compared with the non-aplastic group. RBCs always showed a lower clone size than neutrophils. PNH clone on neutrophils and monocytes was however similar. Inclusion of FLAER increases the sensitivity of the test which is especially useful in picking up small PNH clones in patients of aplastic anemia.
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Favre, Simon; Rougeron, Amandine; Levoir, Laure; Pérard, Baptiste; Milpied, Noël; Accoceberry, Isabelle; Gabriel, Frédéric; Vigouroux, Stéphane
2016-03-01
We report a case of a 27-year old man with severe aplastic anemia who developed a Saprochaete clavata (Geotrichum clavatum) disseminated invasive infection shortly prior a scheduled allogeneic bone marrow transplantation. Treatment with a combination of voriconazole, liposomal amphotericin B and adjuvant granulocyte transfusions was successful before neutrophil recovery.
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Laporte, Joan-Ramon; Vidal, Xavier; Ballarín, Elena; Ibáñez, Luisa
1998-01-01
Aims To determine whether topical ocular chloramphenicol increases the risk of aplastic anaemia and to estimate the magnitude of this risk, if any. Methods Population-based prospective case-control surveillance of aplastic anaemia in a community of 4.2 million inhabitants from 1980 to 1995 (67.2 million person-years) plus case-population estimate of the risk, based on sales figures of ocular chloramphenicol in the study area during the study period. Results One hundred and forty-five patients with aplastic anaemia and 1,226 controls were included in the analysis. Three cases (2.1%) and 5 controls (0.4%) had been exposed to ocular chloramphenicol during the relevant etiological period. The adjusted odds ratio was 3.77 (95% confidence interval, 0.84–16.90). Two cases had also been exposed to other known causes of aplastic anaemia. The incidence of aplastic anaemia among users of ocular chloramphenicol was 0.36 cases per million weeks of treatment. The incidence among non users was 0.04 cases per million weeks. Conclusions An association between ocular chloramphenicol and aplastic anaemia cannot be excluded. However, the risk is less than one per million treatment courses. PMID:9723830
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An unexpected cause for aplastic anaemia in an elderly woman.
Phua, C K; Marimuthu, K; Teo, H Y; Ong, K H; Leo, Y S
2013-02-01
Aplastic anaemia is a rare clinical syndrome associated with diminished or absent precursors in the bone marrow. Acquired aplastic anaemia secondary to human immunodeficiency virus (HIV) is very rare. We present a 71-year-old woman with severe aplastic anaemia secondary to HIV infection, which was after extensive exclusion of other causes. She achieved undetectable viral load after 5 months of combination antiretroviral therapy but remains profoundly pancytopenic, complicated by recurrent infectious and bleeding complications. HIV infection should be considered in patients with pancytopenia.
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Cytogenetic profile of aplastic anaemia in Indian children
Gupta, Vineeta; Kumar, Akash; Saini, Isha; Saxena, Ajit Kumar
2013-01-01
Background & objectives: Aplastic anaemia is a rare haematological disorder characterized by pancytopenia with a hypocellular bone marrow. It may be inherited/genetic or acquired. Acquired aplastic anaemia has been linked to many drugs, chemicals and viruses. Cytogenetic abnormalities have been reported infrequently with acquired aplastic anaemia. Majority of the studies are in adult patients from the West. We report here cytogenetic studies on paediatric patients with acquired aplastic anaemia seen in a tertiary care hospital in north India. Methods: Patients (n=71, age 4-14 yr) were diagnosed according to the guidelines of International Agranulocytosis and Aplastic Anaemia Study. Conventional cytogenetics with Giemsa Trypsin Giemsa (GTG) banding was performed. Karyotyping was done according to the International System for Human Cytogenetics Nomenclature (ISCN). Results: Of the 71 patients, 42 had successful karyotyping where median age was 9 yr; of these 42, 27 (64.3%) patients had severe, nine (21.4%) had very severe and six (14.3%) had non severe aplastic anaemia. Five patients had karyotypic abnormalities with trisomy 12 (1), trisomy 8 (1) and monosomy 7 (1). Two patients had non numerical abnormalities with del 7 q - and t (5:12) in one each. Twenty nine patients had uninformative results. There was no difference in the clinical and haematological profile of patients with normal versus abnormal cytogenetics although the number of patients was small in the two groups. Interpretation & conclusions: Five (11.9%) patients with acquired aplastic anaemia had chromosomal abnormalities. Trisomy was found to be the commonest abnormality. Cytogenetic abnormalities may be significant in acquired aplastic anaemia although further studies on a large sample are required to confirm the findings. PMID:23640556
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Badyal, Rama Kumari; Sachdeva, Man Updesh Singh; Varma, Neelam; Thapa, Babu Ram
2014-01-01
An association between severe aplastic anemia and other autoimmune diseases is rare and has been described in adults for eosinophilic fasciitis, thymomas, systemic lupus erythematosus, and thyroid disorders. Herein we report a patient with celiac disease who was not strictly following a gluten-free diet and presented with progressive pallor, fever, and weakness of 1 month's duration. On investigation, he had pancytopenia, which on subsequent evaluation revealed aplastic anemia. An association between aplastic anemia and celiac disease has rarely been reported. To the best of author's knowledge, only 1 pediatric case of celiac disease associated with aplastic anemia has been published. This is the second report to suggest such an association in children.
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Usuki, Kensuke
2016-01-01
Treatments of aplastic anemia are comprised of supportive therapy and aplastic anemia-specific therapy aimed at restoring hematopoiesis. Supportive therapies include transfusion, G-CSF, and the administration of iron chelation agents, as well as dealing specifically with individual symptoms. Aplastic anemia-specific treatments given with the aim of achieving hematopoietic recovery include immunosuppressive therapy, allogeneic hematopoietic stem cell transplantation, and anabolic hormone therapy. Although transplantation provides complete recovery of hematopoiesis (cure), there is a risk of death due to transplant-related complications. The most effective immunosuppressive therapy is a combination of anti-thymocyte globulin and cyclosporine. This treatment is also effective against the secondary, drug-induced and hepatitis-associated forms of aplastic anemia. In the management of aplastic anemia, a treatment is selected from among these options depending on the disease severity and the age of the individual case. The thrombopoietin receptor agonist eltrombopag appears to be effective and to provide tri-lineage recovery of hematopoiesis in some cases. Indications for its use are expected to expand in Japan.
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Development of clinical paroxysmal nocturnal haemoglobinuria in children with aplastic anaemia.
Narita, Atsushi; Muramatsu, Hideki; Okuno, Yusuke; Sekiya, Yuko; Suzuki, Kyogo; Hamada, Motoharu; Kataoka, Shinsuke; Ichikawa, Daisuke; Taniguchi, Rieko; Murakami, Norihiro; Kojima, Daiei; Nishikawa, Eri; Kawashima, Nozomu; Nishio, Nobuhiro; Hama, Asahito; Takahashi, Yoshiyuki; Kojima, Seiji
2017-09-01
The clinical significance of paroxysmal nocturnal haemoglobinuria (PNH) in children with aplastic anaemia (AA) remains unclear. We retrospectively studied 57 children with AA between 1992 and 2010. During the follow-up, five patients developed clinical PNH, in whom somatic PIGA mutations were detected by targeted sequencing. The 10-year probability of clinical PNH development was 10·2% (95% confidence interval, 3·6-20·7%). Furthermore, the detection of minor PNH clones by flow cytometry at AA diagnosis was a risk factor for the subsequent development of clinical PNH. These patients with PNH clones at AA diagnosis should undergo periodic monitoring for potential clinical PNH development. © 2017 John Wiley & Sons Ltd.
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Bueno, Clara; Roldan, Mar; Anguita, Eduardo; Romero-Moya, Damia; Martín-Antonio, Beatriz; Rosu-Myles, Michael; del Cañizo, Consuelo; Campos, Francisco; García, Regina; Gómez-Casares, Maite; Fuster, Jose Luis; Jurado, Manuel; Delgado, Mario; Menendez, Pablo
2014-07-01
Aplastic anemia is a life-threatening bone marrow failure disorder characterized by peripheral pancytopenia and marrow hypoplasia. The majority of cases of aplastic anemia remain idiopathic, although hematopoietic stem cell deficiency and impaired immune responses are hallmarks underlying the bone marrow failure in this condition. Mesenchymal stem/stromal cells constitute an essential component of the bone marrow hematopoietic microenvironment because of their immunomodulatory properties and their ability to support hematopoiesis, and they have been involved in the pathogenesis of several hematologic malignancies. We investigated whether bone marrow mesenchymal stem cells contribute, directly or indirectly, to the pathogenesis of aplastic anemia. We found that mesenchymal stem cell cultures can be established from the bone marrow of aplastic anemia patients and display the same phenotype and differentiation potential as their counterparts from normal bone marrow. Mesenchymal stem cells from aplastic anemia patients support the in vitro homeostasis and the in vivo repopulating function of CD34(+) cells, and maintain their immunosuppressive and anti-inflammatory properties. These data demonstrate that bone marrow mesenchymal stem cells from patients with aplastic anemia do not have impaired functional and immunological properties, suggesting that they do not contribute to the pathogenesis of the disease. Copyright© Ferrata Storti Foundation.
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Aplastic anemia as a feature of systemic lupus erythematosus: a case report and literature review.
Chalayer, Émilie; Ffrench, Martine; Cathébras, Pascal
2015-06-01
Peripheral cytopenias are common in systemic lupus erythematosus, but bone marrow involvement is rarely reported. Aplastic anemia is the result of immune-mediated destruction of hematopoietic stem cells causing pancytopenia and characterized by an empty bone marrow. This rare but serious disease has been described as an unusual manifestation of systemic lupus erythematosus. We reviewed the 25 cases published in the English language literature and discuss the clinical presentation, outcome, treatment, and pathophysiology of aplastic anemia as a complication of systemic lupus erythematosus. We report here the first case of aplastic anemia associated with systemic lupus erythematosus treated with an allogeneic hematopoietic stem cell transplant. Over one half of patients received concomitantly the diagnoses of systemic lupus erythematosus and aplastic anemia. No clinical or histological features can distinguish primary aplastic anemia from aplastic anemia occurring in systemic lupus erythematosus patients. The overall mortality is about 15% and corticosteroid-based therapy alone or in combination with other immunomodulatory drugs can restore bone marrow function. Systemic lupus erythematosus may be complicated by bone marrow involvement. The diagnosis of peripheral cytopenias should be confirmed by bone marrow aspiration. All these patients should receive cortisone as a first treatment. Plasma exchanges seem to have some efficacy. Other different immunomodulatory therapies were used with variable results.
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Interleukin-6 and Interleukin-8 Levels Correlate With the Severity of Aplastic Anemia in Children.
Gupta, Vineeta; Kumar, Sushil; Sonowal, Rimjhim; Singh, Surya K
2017-04-01
The aim of this study was to evaluate the levels of interleukin (IL)-6 and IL-8 in patients with aplastic anemia and its correlation with severity of the disease. IL-6 and IL-8 levels were measured in 40 patients with aplastic anemia in the age group of 4 to 14 years. A total of 40 healthy children served as controls. Quantitative estimation of IL-6 and IL-8 was performed using a solid-phase sandwich ELISA kit. Results were presented as IL-6 and IL-8 concentrations in pg/mL. Patients received immunosuppressive therapy per the British Committee for Standards in Haematology Guidelines 2009. Mean age of the patients was 9.78±2.74 years. IL-6 level of patients was elevated compared with controls (193.48±352.3 vs. 4.58±3.39; P<0.001). IL-8 levels were also significantly elevated in patients compared with controls (15.58±18.0 vs. 1.85±0.95; P<0.001). IL levels were also assessed in relation to severity of the disease. Levels were the highest in patients with very severe aplastic anemia (724.33±519.42), followed by severe aplastic anemia (80.51±66.28 pg/mL), and non-severe aplastic anemia (6.01±1.89). Differences were statistically significant. A similar trend was also observed for IL-8 levels, where the levels were 41.02±24.23, 11.34±8.0, and 1.67±0.71 for very severe aplastic anemia, severe aplastic anemia, and non-severe aplastic anemia, respectively. The differences were again statistically significant. IL levels were also correlated with the treatment outcome. Responders had lower levels compared with nonresponders, but the difference was not statistically significant (186.36±322.45 vs. 198.74±368.10). Levels of ILs decreased in responders, but were not comparable with that of controls 6 months after therapy. High levels of IL-6 and IL-8 were observed in children with aplastic anemia. Increased levels showed correlation with disease severity and therefore appear to play an important role in aplastic anemia. However, levels had no significant correlation with the treatment outcome.
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[Long-term Follow-up of Patients with Hepatitis-Associated Aplastic Anemia].
You, Ya-Hong; Meng, Xian-Bin; Li, Xing-Xin; Ge, Mei-Li; Nie, Neng; Huang, Jin-Bo; Zhang, Jing; Huang, Zhen-Dong; Shao, Ying-Qi; Shi, Jun; Zheng, Yi-Zhou
2017-08-01
To explore the clinical characteristic, therapeutic efficacy and prognosis of patients with hepatitis-associated aplasitc anemia (HAAA). the clinical data and labrotatory examination results of 30 cases of HAAA were analyzed retrospectively, the 6-month response ratio and overall survival (OS) were assessed. HAAA most commonly occured in males, with the occurence rate of males and females was 4:1, the median onset age was 16 (4-43) years old, HAAA oriented focus on sever aplastic anemia (SAA)(4 cases,13%) and very sever aplastic anemia (VSAA)(22 cases,73%). Aplastic anemia (AA) could be seen on occurence of hepatitis (accompanied aplastic anemia) (7 cases,23%), or after the onset of hepatits (delayed aplastic anemia) (23 cases,77%), but more often occured in the latter. Statistical analysis showed that when compared with the patients of delayed aplastic anemia, patients accompanied aplastic anemia possesses lower levels of glutamic-pyruvic transaminase(ALT), aspertate aminotransferase (AST) and total bilirubin (TBIL)(P=0.042,0.012,0.001), and possessed a more obvious lymphoid cell disorder when AA occured, with more lower peripheral blood CD19 + B cells proportion (P=0.046) and more obvious imbalance of CD4 + /CD8 + ratio, but the difference was no statistical significant (P=0538). Factors affecting the 6-month respose were the severity of AA (P=0.044), the peak level of bilirubin of hepatitis (P=0.006) and the propotion of mature monocyte in bone marrow (P=0.034). The long-term follow-up showed that the 2-year OS of HAAA was 64.3±9.2%, the 6-month curative efficacy significantly affect the prognosis (P<0.001). HAAA more often occur in young male, HAAA is mainly SAA and VSAA and mostly non-A-C hepatitis associated aplastic anemia, patients usually have a high incidence of early infection. Patients acompanied with aplastic anemia possess more obvious immunological derangement; the treatment efficacy for HAAA is poor, patients who haven't obtained 6-month response indicate a sinister prognosis, allogeneic hematopoietic stem cell transplantion is a better choice for these patients.
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Resolution of acute hepatitis B-associated aplastic anaemia with antiviral therapy.
Hendren, Nicholas; Moore, Joseph; Hofmann, Sandra; Rambally, Siayareh
2017-10-03
A previously healthy 44-year-old woman presented with 3 days of worsening petechial rash, epistaxis and fatigue. Admission labs revealed pancytopenia, low reticulocyte index and elevated liver enzymes. Bone marrow biopsy demonstrated a profoundly hypocellular bone marrow without dysplasia and additional testing demonstrated an acute hepatitis B infection. In the context of an acute hepatitis B infection, elevated liver enzymes and aplastic anaemia, our patient was diagnosed with severe hepatitis-associated aplastic anaemia due to an acute hepatitis B infection. She was initiated on antiviral therapy with tenofovir and briefly received immunosuppressive therapy with a robust sustained improvement in her blood counts. Acute hepatitis B-associated aplastic anaemia is an exceptionally rare presentation of aplastic anaemia. We present acute hepatitis B-associated aplastic anaemia that resolved with antiviral therapy, which to our knowledge is the second such case reported in the literature and the first using tenofovir. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
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Bär, Christian; Huber, Nicolas; Beier, Fabian; Blasco, Maria A.
2015-01-01
Aplastic anemia is a rare but life-threatening disorder characterized by cytopenia in at least two of the three blood lineages. A frequent feature of patients with aplastic anemia is that they have shorter telomeres than those of age-matched controls. Testosterone has been used for over half a century in the treatment of aplastic anemia. However, although remissions are frequent following hormone therapy, the molecular mechanism underlying the response to treatment has remained unknown. Here we explored the possibility that the recently described regulation of telomerase activity by sex hormones may be the mechanism responsible. To this end, we used a mouse model of aplastic anemia induced by short telomeres in the bone marrow compartment. We found that testosterone therapy results in telomerase up-regulation, improved blood counts, and a significant extension of life-span of these mice. Importantly, longitudinal follow-up studies revealed longer telomeres in peripheral blood in mice subjected to hormone treatment. Our results demonstrate that testosterone-mediated telomerase activation can attenuate or reverse aplastic anemia disease progression associated with the presence of short telomeres. PMID:26206796
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Aplastic anemia, mucormycosis and aspergillosis in infectious mononucleosis: success is possible.
Ergene, Ulku; Ozbalci, Demircan; Baykal, Bahattin; Metin Ciris, I; Yariktas, Murat
2007-10-01
Infectious mononucleosis (IM) is a rare cause of aplastic anemia in adults. We report of a patient in whom aplastic anemia, mucormycosis and aspergillosis complicated during the course of IM and successfully treated with liposomal amphotericin B. According to our searches in literature, we could not find a similar patient complicated and successfully treated like ours.
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Desmond, Ronan; Dunbar, Cynthia E.; Young, Neal S.
2014-01-01
Aplastic anemia is a bone marrow failure syndrome that causes pancytopenia and can lead to life-threatening complications. Bone marrow transplantation remains the standard of care for younger patients and those with a good performance status but many patients may not have a suitable donor. Immunosuppressive therapy is able to resolve cytopenias in a majority of patients with aplastic anemia but relapses are not uncommon and some patients remain refractory to this approach. Patients may require frequent blood and platelet transfusion support which is expensive and inconvenient. Life-threatening bleeding complications still occur despite prophylactic platelet transfusion. Thrombopoietin (TPO) mimetics, such as romiplostim and eltrombopag, were developed to treat patients with refractory immune thrombocytopenia but are now being investigated for the treatment of bone marrow failure syndromes. TPO is the main regulator for platelet production and its receptor (c-Mpl) is present on megakaryocytes and hematopoietic stem cells. Trilineage hematopoietic responses were observed in a recent clinical trial using eltrombopag in patients with severe aplastic anemia refractory to immunosuppression suggesting that these agents can provide a new therapeutic option for enhancing blood production. In this review, we discuss these recent results and ongoing investigation of TPO mimetics for aplastic anemia and other bone marrow failure states like myelodysplastic syndromes. Clonal evolution or progression to acute myeloid leukemia remains a concern when using these drugs in bone marrow failure and patients should only be treated in the setting of a clinical trial. PMID:23690288
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Yoshimi, Ayami; Strahm, Brigitte; Baumann, Irith; Furlan, Ingrid; Schwarz, Stephan; Teigler-Schlegel, Andrea; Walther, Joachim-Ulrich; Schlegelberger, Brigitte; Göhring, Gudrun; Nöllke, Peter; Führer, Monika; Niemeyer, Charlotte M
2014-03-01
Secondary myelodysplastic syndrome and acute myelogenous leukemia (sMDS/sAML) are the most serious secondary events occurring after immunosuppressive therapy in patients with aplastic anemia. Here we evaluate the outcome of hematopoietic stem cell transplantation (HSCT) in 17 children and young adults with sMDS/sAML after childhood aplastic anemia. The median interval between the diagnosis of aplastic anemia and the development of sMDS/sAML was 2.9 years (range, 1.2 to 13.0 years). At a median age of 13.1 years (range, 4.4 to 26.7 years), patients underwent HSCT with bone marrow (n = 6) or peripheral blood stem cell (n = 11) grafts from HLA-matched sibling donors (n = 2), mismatched family donors (n = 2), or unrelated donors (n = 13). Monosomy 7 was detected in 13 patients. The preparative regimen consisted of busulfan, cyclophosphamide, and melphalan in 11 patients and other agents in 6 patients. All patients achieved neutrophil engraftment. The cumulative incidence of grade II-IV acute graft-versus-host disease (GVHD) was 47%, and that of chronic GVHD was 70%. Relapse occurred in 1 patient. The major cause of death was transplant-related complication (n = 9). Overall survival and event-free survival at 5 years after HSCT were both 41%. In summary, this study indicates that HSCT is a curative therapy for some patients with sMDS/sAML after aplastic anemia. Future efforts should focus on reducing transplantation-related mortality. Copyright © 2014 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.
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Epidemiology of aplastic anemia: a prospective multicenter study.
Montané, Eva; Ibáñez, Luisa; Vidal, Xavier; Ballarín, Elena; Puig, Ramon; García, Nuria; Laporte, Joan-Ramon
2008-04-01
Aplastic anemia is a rare and severe disease. Its incidence varies considerably worldwide. We aimed at describing the epidemiology of this disease, including the incidence, mortality and survival trends, in a well-defined population. Since 1980, a case-control surveillance study of aplastic anemia has been carried out by a cooperative group, in the metropolitan area of Barcelona. Inclusion is dependent on the patient having at least two of the following features: white blood cell count < or = 3.5 x 10(9)/L, platelet count < or = 50 x 10(9)/L, hemoglobin <10 g/L or hematocrit of <30%; when only one of these last two criteria is fulfilled, a reticulocyte count of < or = 30 x 10(9)/L is also required. The bone marrow biopsy has to be compatible with the diagnosis of aplastic anemia. Between 1980 and 2003, a total of 235 cases of aplastic anemia were identified. The overall incidence was 2.34 per million inhabitants per year and the incidence increased with age. Most of the cases were classified as severe or very severe aplastic anemia. Survival rates at 3 months, and at 2 and 15 years after the diagnosis were 73%, 57%, and 51%, respectively. Advanced age and more severe disease at the time of diagnosis were associated with a lower survival rate. There was a trend to a better 2-year survival rate among patients treated with bone marrow transplantation. Forty-nine cases (20.8%) were exposed to drugs reported to be associated with aplastic anemia, and 21 (8.9%) to toxic agents. The incidence of aplastic anemia in Barcelona is low but the case fatality rate is high. Advanced age and severe disease at the time of diagnosis were associated with decreased survival.
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[Avascular necrosis of the femoral head in a patient with severe aplastic anaemia].
Plichta, Piotr; Pawelec, Katarzyna
2011-01-01
Avascular necrosis (AVN) is a pathological condition associated with numerous processes. Most frequent causes of ischaemia of the femoral head include trauma, corticosteroid therapy, radiation therapy, alcoholism, Gaucher's disease, systemic lupus erythematosus, rheumatoid arthritis. Corticosteroid therapy is considered the most frequent risk factor for developing avascular necrosis. In this paper we report a case of a 19-year old female patient who developed avascular necrosis of the right femoral head following treatment of severe aplastic anaemia. Clinical symptoms included groin pain and fever, painful abduction and internal rotation, tenderness over the right hip joint. The plain X-ray was not specific. However magnetic resonance imaging of the hip revealed changes characteristic for avascular necrosis. Conservative treatment was administrated. Two years after the onset of first clinical symptoms the patient is able to walk without pain, although the range of motion of the right hip joint is partially diminished. We evaluated the possible risk factors of AVN, diagnostic methods and prognosis. The onset of avascular necrosis should be considered as one of the relevant complications in patients with severe aplastic anaemia following immunosuppressive treatment.
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Bär, Christian; Huber, Nicolas; Beier, Fabian; Blasco, Maria A
2015-10-01
Aplastic anemia is a rare but life-threatening disorder characterized by cytopenia in at least two of the three blood lineages. A frequent feature of patients with aplastic anemia is that they have shorter telomeres than those of age-matched controls. Testosterone has been used for over half a century in the treatment of aplastic anemia. However, although remissions are frequent following hormone therapy, the molecular mechanism underlying the response to treatment has remained unknown. Here we explored the possibility that the recently described regulation of telomerase activity by sex hormones may be the mechanism responsible. To this end, we used a mouse model of aplastic anemia induced by short telomeres in the bone marrow compartment. We found that testosterone therapy results in telomerase up-regulation, improved blood counts, and a significant extension of life-span of these mice. Importantly, longitudinal follow-up studies revealed longer telomeres in peripheral blood in mice subjected to hormone treatment. Our results demonstrate that testosterone-mediated telomerase activation can attenuate or reverse aplastic anemia disease progression associated with the presence of short telomeres. Copyright© Ferrata Storti Foundation.
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Epidemiology, Clinico-Haematological Profile and Management of Aplastic Anaemia: AIIMS Experience.
Mahapatra, M; Singh, P K; Agarwal, M; Prabhu, M; Mishra, P; Seth, T; Tyagi, S; Patil, H P; Saxena, R
2015-03-01
The incidence of aplastic anaemia (AA) is higher in Asia than in the West. The precise incidence of AA in India is not known due to lack of epidemiological study. 20-40% of pancytopenic patients in referral centres are of aplastic anaemia. This was an analysis of 1501 patients diagnosed with aplastic anaemia over a period of seven and half years (January 2007- June 2014) attending the Aplastic clinic of department of haematology of All India Institute of Medical Sciences, New Delhi. The details regarding medical history, physical examination, complete blood count, bone marrow aspirate and biopsy, treatment received, were retrieved. Inherited bone marrow failure was screened in patients below 35 years. Treatment response was analysed for various treatment modalities. 1501 patients of AA from 20 different states of India were analysed. The bulk of patients were from Uttar Pradesh (28.7%), Bihar (23.6%), Delhi/NCR (20%) and Haryana (7%).The average number of new aplastic anaemia patients enrolled per year 214 (range: 101 -263). The median age at presentation was 25 years (range 2-83),with M;F - 2.3:1. Severity of AA revealed: severe (SAA): 75%, very severe (VSAA): 15%, non-severe (NSAA): 10%. Inherited bone marrow failure syndromes constituted 5% (75 patients) of all aplastic anaemia patients. The most common clinical presentations were pallor (97%), bleeding manifestations (69.6%) and fever (54%). The haematological parameters showed: median level of haemoglobin level: 5.9 gm/dL, WBC: 2700/mm3, ANC: 380/mm3, platelet: 1 0000/mm3. PNH clone was present in 13.5% of patients. 107 patients (7%) were lost to follow up or expired before any treatment was initiated. Only 69 patients (4.5%) received treatment with HLA-matched sibling stem cell transplantation and another 232 (15.5%) patients received ATG plus cyclosporine as immunosuppressive therapy. Seven hundred thirteenpatients (47.5%) received cyclosporine. The overall response to various treatment modalities was: HLA matched sibling haematopoietic stem cell transplant: 75.3%, Anti-thymocyte globulin plus cyclosporine: 58.7%, cyclosporine plus androgen: 45.6%, cyclosporine alone: 32.2%. Management of AA is a real challenge in developing countries.This is one of the largest case series from a single centre from India. It is our endeavour to reduce the detrimental outcome by increasing awareness among patients and referring physicians to reduce the delay between diagnosis and treatment.
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Zeng, Qiang; Chang, Hong
2016-10-01
To investigate the correlation of single nucleotide polymorphism (SNP) of Interleukin-2(IL-2)-330T/G with genetic susceptibility and the efficacy of immunosuppressive therapy in patients with aplastic anemia. The peripheral blood samples from 103 patients with aplastic anemia in our hospital were collected. Out of 103 patients 46 received immuosuppressive therapy and were observed for 4 months, and 100 healthy adults were selected as control. The electrophoresis and DNA sequence were performed. The polymerase chain reaction(PCR) was used to amplify the polymorphic gene segment of IL-2 -330T/G from 103 aplastic anemia patients and 100 healthy adults. The frequencis of IL-2-330 GG genotype and G allele were a little higher in patients with aplastic anemia than that in the healthy adults(12.6% vs 12.0%, P>0.05; 27.7% vs 33.5%, P>0.05), but not statistically significant(P>0.05); in the 103 patients with aplastic anemia, 46 received immunosuppressive therapy, whereas 29 patients showed response, no significant difference was found between the responders and non-responders in the IL-2-330 GG genotype and G allele (31.0% vs 48.3%, P>0.05; 64.8% vs 61.8%, P>0.05). IL-2 -330T/G gene polymorphism may not correlate with the susceptibility of aplastic anemia or the efficacy of immunosuppressive therapy.
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McCabe, Amanda; Smith, Julianne N P; Costello, Angelica; Maloney, Jackson; Katikaneni, Divya; MacNamara, Katherine C
2018-05-17
Severe aplastic anemia results from profound hematopoietic stem cell loss. T cells and interferon gamma have long been associated with severe aplastic anemia, yet the underlying mechanisms driving hematopoietic stem cell loss remain unknown. Using a mouse model of severe aplastic anemia, we demonstrate that interferon gamma-dependent hematopoietic stem cell loss required macrophages. Interferon gamma was necessary for bone marrow macrophage persistence, despite loss of other myeloid cells and hematopoietic stem cells. Depleting macrophages or abrogating interferon gamma signaling specifically in macrophages did not impair T cell activation or interferon gamma production in the bone marrow but rescued hematopoietic stem cells and reduced mortality. Thus, macrophages are not required for induction of interferon gamma in severe aplastic anemia and rather act as sensors of interferon gamma. Macrophage depletion rescued thrombocytopenia, increased bone marrow megakaryocytes, preserved platelet-primed stem cells, and increased the platelet-repopulating capacity of transplanted hematopoietic stem cells. In addition to the hematopoietic effects, severe aplastic anemia induced loss of non-hematopoietic stromal populations, including podoplanin-positive stromal cells. However, a subset of podoplanin-positive macrophages was increased during disease, and blockade of podoplanin in mice was sufficient to rescue disease. Our data further our understanding of disease pathogenesis demonstrating a novel role for macrophages as sensors of interferon gamma, thus illustrating an important role for the microenvironment in pathogenesis of severe aplastic anemia. Copyright © 2018, Ferrata Storti Foundation.
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Aplastic crisis in occult hereditary spherocytosis caused by human parvovirus (HPV B19).
Rappaport, E S; Quick, G; Ransom, D; Helbert, B; Frankel, L S
1989-02-01
We have reported a case of aplastic crisis occurring in an 11-year-old black boy with occult hereditary spherocytosis. An etiologic diagnosis of human parvovirus (HPV) B19 infection was confirmed serologically. The Coulter Model S + IV proved useful for both diagnosis and treatment monitoring through serial histograms. The relationship of HPV infection and aplastic crisis is discussed.
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Somatic Mutations and Clonal Hematopoiesis in Aplastic Anemia.
Yoshizato, Tetsuichi; Dumitriu, Bogdan; Hosokawa, Kohei; Makishima, Hideki; Yoshida, Kenichi; Townsley, Danielle; Sato-Otsubo, Aiko; Sato, Yusuke; Liu, Delong; Suzuki, Hiromichi; Wu, Colin O; Shiraishi, Yuichi; Clemente, Michael J; Kataoka, Keisuke; Shiozawa, Yusuke; Okuno, Yusuke; Chiba, Kenichi; Tanaka, Hiroko; Nagata, Yasunobu; Katagiri, Takamasa; Kon, Ayana; Sanada, Masashi; Scheinberg, Phillip; Miyano, Satoru; Maciejewski, Jaroslaw P; Nakao, Shinji; Young, Neal S; Ogawa, Seishi
2015-07-02
In patients with acquired aplastic anemia, destruction of hematopoietic cells by the immune system leads to pancytopenia. Patients have a response to immunosuppressive therapy, but myelodysplastic syndromes and acute myeloid leukemia develop in about 15% of the patients, usually many months to years after the diagnosis of aplastic anemia. We performed next-generation sequencing and array-based karyotyping using 668 blood samples obtained from 439 patients with aplastic anemia. We analyzed serial samples obtained from 82 patients. Somatic mutations in myeloid cancer candidate genes were present in one third of the patients, in a limited number of genes and at low initial variant allele frequency. Clonal hematopoiesis was detected in 47% of the patients, most frequently as acquired mutations. The prevalence of the mutations increased with age, and mutations had an age-related signature. DNMT3A-mutated and ASXL1-mutated clones tended to increase in size over time; the size of BCOR- and BCORL1-mutated and PIGA-mutated clones decreased or remained stable. Mutations in PIGA and BCOR and BCORL1 correlated with a better response to immunosuppressive therapy and longer and a higher rate of overall and progression-free survival; mutations in a subgroup of genes that included DNMT3A and ASXL1 were associated with worse outcomes. However, clonal dynamics were highly variable and might not necessarily have predicted the response to therapy and long-term survival among individual patients. Clonal hematopoiesis was prevalent in aplastic anemia. Some mutations were related to clinical outcomes. A highly biased set of mutations is evidence of Darwinian selection in the failed bone marrow environment. The pattern of somatic clones in individual patients over time was variable and frequently unpredictable. (Funded by Grant-in-Aid for Scientific Research and others.).
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Giudice, Valentina; Feng, Xingmin; Lin, Zenghua; Hu, Wei; Zhang, Fanmao; Qiao, Wangmin; Ibanez, Maria Del Pilar Fernandez; Rios, Olga; Young, Neal S
2018-05-01
Oligoclonal expansion of CD8 + CD28 - lymphocytes has been considered indirect evidence for a pathogenic immune response in acquired aplastic anemia. A subset of CD8 + CD28 - cells with CD57 expression, termed effector memory cells, is expanded in several immune-mediated diseases and may have a role in immune surveillance. We hypothesized that effector memory CD8 + CD28 - CD57 + cells may drive aberrant oligoclonal expansion in aplastic anemia. We found CD8 + CD57 + cells frequently expanded in the blood of aplastic anemia patients, with oligoclonal characteristics by flow cytometric Vβ usage analysis: skewing in 1-5 Vβ families and frequencies of immunodominant clones ranging from 1.98% to 66.5%. Oligoclonal characteristics were also observed in total CD8 + cells from aplastic anemia patients with CD8 + CD57 + cell expansion by T-cell receptor deep sequencing, as well as the presence of 1-3 immunodominant clones. Oligoclonality was confirmed by T-cell receptor repertoire deep sequencing of enriched CD8 + CD57 + cells, which also showed decreased diversity compared to total CD4 + and CD8 + cell pools. From analysis of complementarity-determining region 3 sequences in the CD8 + cell pool, a total of 29 sequences were shared between patients and controls, but these sequences were highly expressed in aplastic anemia subjects and also present in their immunodominant clones. In summary, expansion of effector memory CD8 + T cells is frequent in aplastic anemia and mirrors Vβ oligoclonal expansion. Flow cytometric Vβ usage analysis combined with deep sequencing technologies allows high resolution characterization of the T-cell receptor repertoire, and might represent a useful tool in the diagnosis and periodic evaluation of aplastic anemia patients. (Registered at clinicaltrials.gov identifiers: 00001620, 01623167, 00001397, 00071045, 00081523, 00961064 ). Copyright © 2018 Ferrata Storti Foundation.
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Hepatitis Associated Aplastic Anemia: A review
2011-01-01
Hepatitis-associated aplastic anemia (HAAA) is an uncommon but distinct variant of aplastic anemia in which pancytopenia appears two to three months after an acute attack of hepatitis. HAAA occurs most frequently in young male children and is lethal if leave untreated. The etiology of this syndrome is proposed to be attributed to various hepatitis and non hepatitis viruses. Several hepatitis viruses such as HAV, HBV, HCV, HDV, HEV and HGV have been associated with this set of symptoms. Viruses other than the hepatitis viruses such as parvovirus B19, Cytomegalovirus, Epstein bar virus, Transfusion Transmitted virus (TTV) and non-A-E hepatitis virus (unknown viruses) has also been documented to develop the syndrome. Considerable evidences including the clinical features, severe imbalance of the T cell immune system and effective response to immunosuppressive therapy strongly present HAAA as an immune mediated mechanism. However, no association of HAAA has been found with blood transfusions, drugs and toxins. Besides hepatitis and non hepatitis viruses and immunopathogenesis phenomenon as causative agents of the disorder, telomerase mutation, a genetic factor has also been predisposed for the development of aplastic anemia. Diagnosis includes clinical manifestations, blood profiling, viral serological markers testing, immune functioning and bone marrow hypocellularity examination. Patients presenting the features of HAAA have been mostly treated with bone marrow or hematopoietic cell transplantation from HLA matched donor, and if not available then by immunosuppressive therapy. New therapeutic approaches involve the administration of steroids especially the glucocorticoids to augment the immunosuppressive therapy response. Pancytopenia following an episode of acute hepatitis response better to hematopoietic cell transplantation than immunosuppressive therapy. PMID:21352606
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The impact of Asian descent on the incidence of acquired severe aplastic anaemia in children.
McCahon, Emma; Tang, Keith; Rogers, Paul C J; McBride, Mary L; Schultz, Kirk R
2003-04-01
Previous studies have suggested an increased incidence of acquired severe aplastic anaemia in Asian populations. We evaluated the incidence of aplastic anaemia in people of Asian descent, using a well-defined paediatric (0-14 years) population in British Columbia, Canada to minimize environmental factors. The incidence in children of East/South-east Asian descent (6.9/million/year) and South Asian (East Indian) descent (7.3/million/year) was higher than for those of White/mixed ethnic descent (1.7/million/year). There appeared to be no contribution by environmental factors. This study shows that Asian children have an increased incidence of severe aplastic anaemia possibly as a result of a genetic predisposition.
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Zimmer, B; Guitard, Y
2001-09-01
A method is presented that allows unilateral space closure in patients with aplastic lower second premolars. Based on a straight-wire appliance, space closure was achieved with a combination of "push mechanics" using the second molar as an anchorage unit and Class II "pull mechanics", thus avoiding the application of any distalizing force on the lower incisors. The results from 13 consecutively treated subjects (five boys, eight girls, mean age 12 years and 6 months) were analyzed. Complete bodily space closure was achieved in all 13 cases within a mean treatment time of 2 years and 7 months. The desired Angle Class III molar relationship of one premolar width (+/- 1/4 premolar width) on the aplastic side was successful in eleven patients, an additional 4.7 mm of space being created for the third molar on the aplastic side compared with the contralateral side (p < or = 0.01). However, adverse effects could be kept to a minimum, with no method-dependent side effects being recorded with regard to canine and molar relationships on the contralateral side, or to overbite, overjet, or upper and lower incisor inclination. The mean lower midline shift of 0.8 mm was in accordance with the mean distal canine relationship of 1/3 premolar width on the aplastic side. These results confirm that orthodontic space closure in cases of unilateral aplastic lower second premolars can be performed successfully with the presented treatment method without the need for additional premolar extractions, prosthodontic treatment or implants. Furthermore, the prognosis for the lower wisdom tooth on the aplastic side is improved.
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Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make ... blood cells. There are different types, including Fanconi anemia. Causes include Toxic substances, such as pesticides, arsenic, ...
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... sudden blood loss, long-term diseases, kidney failure , aplastic anemia , or man-made heart valves). MCV above normal. ... sudden blood loss, long-term diseases, kidney failure, aplastic anemia, or man-made heart valves). MCH above normal. ...
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Aplastic Anemia & MDS International Foundation
... Seychelles Sierra Leone Singapore Sint Maarten Slovakia Slovenia Solomon Islands Somalia South Africa South Georgia and the ... Tribute Of Individual Gifts Corporate Sponsorship Invest in Research Diseases Aplastic Anemia Causes Symptoms Diagnosis Types Treatments ...
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Management of aplastic anaemia in pregnancy in a resource poor centre.
John, Celestine Osita; Korubo, Kaladada; Ogu, Rosemary; Mmom, Chigozirim Faith; Mba, Alpheus Gogo; Chidiadi, Ezenwa-Ahanene; Akani, Chris
2016-01-01
Aplastic anaemia occurring in pregnancy is a rare event with life threatening challenges for both mother and child. We present a successful fetomaternal outcome despite the challenges in the management of this rare condition in a tertiary but resource poor centre. This is case of a 37 year old Nigerian woman G6P0 +5 managed with repeated blood transfusions from 28 weeks of gestation for bone marrow biopsy confirmed aplastic anaemia following presentation with weakness and gingival bleeds. She had a cesarean section at 37 weeks for pre-eclampsia and oligohydraminous with good feto-maternal outcome. She was managed entirely with fresh whole blood and received 21 units. Aplastic Anaemia in Pregnancy is a rare event with poor feto maternal prognosis. Successful management is possible with good multi-disciplinary approach and availability of supportive comprehensive obstetric care.
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Remission of aplastic anemia induced by treatment for Graves disease in a pediatric patient.
Das, Prabodh Kumar; Wherrett, Diane; Dror, Yigal
2007-08-01
Aplastic anemia (AA) is mediated by T-cell autoimmunity in the majority of cases; it is rare and mostly idiopathic in children. We describe a child, who developed AA following Graves' disease which could not be attributed to antithyroid drugs. We hypothesized that both diseases were caused by similar autoimmune process. We monitored the blood counts and did not administer any conventional treatment for AA assuming that the existing anti- hematopoietic stem cell humoral and cellular immunity might subside with induction of remission of Grave's disease. The child went into complete remission with the treatment of the Graves' disease.
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Blauel, Emily R; Grossmann, Lily T; Vissa, Madhav; Miller, Scott T
2015-10-01
In a patient with sickle cell disease receiving chronic transfusion, exacerbation of anemia with reticulocytopenia must prompt consideration of a delayed hemolytic transfusion reaction with hyperhemolysis, as further transfusion may worsen this condition; definitive diagnosis is sometimes difficult. Anemia evolving during parvovirus B19-induced erythroid hypoplasia (transient aplastic crisis) should be attenuated in chronic transfusion patients due to superior survival of transfused over endogenous red blood cells. A 16-year-old with sickle cell disease receiving chronic transfusion of modified intensity (goal to maintain hemoglobin S<50%) who developed symptomatic anemia with reticulocytopenia was later shown to have had transient aplastic crisis.
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Features of Hepatitis in Hepatitis-associated Aplastic Anemia: Clinical and Histopathologic Study.
Patel, Kalyani R; Bertuch, Alison; Sasa, Ghadir S; Himes, Ryan W; Wu, Hao
2017-01-01
Hepatitis-associated aplastic anemia (HAA) is a rare variant of aplastic anemia in which patients present with severe pancytopenia after an episode of acute hepatitis. The marrow failure is often rapid, severe, and usually fatal if untreated. The preceding hepatitis is largely under-studied. Retrospective study of the clinical and histopathologic features of hepatitis in pediatric patients who subsequently developed aplastic anemia and comparison with consecutive cases of acute liver failure and random cases of autoimmune hepatitis during the same time frame. All 7 patients of HAA had significant elevations in aminotransferases and conjugated hyperbilirubinemia at initial presentation. Echoing liver function indices, cholestatic hepatitis with sinusoidal obstruction-type endothelial injury was seen histomorphologically. Autoimmune hepatitis serology such as anti-F-actin, anti-liver/kidney microsome, and hypergammaglobulinemia was negative in all patients. Five of 7 patients (71.4%) had, however, elevated antinuclear antibody, all with a speckled pattern. Hepatitis virus serology was negative in all patients. By immunohistochemical staining, the lobular CD8/CD4 lymphocyte ratio was markedly elevated in all of the initial samples with significant reduction in this ratio (P = 0.03) in 3 patients post treatment (ursodiol, antibiotics, and/or immunosuppressive therapy). Hepatitis preceding HAA is characterized by marked elevation of aminotransferases, conjugated hyperbilirubinemia, elevated antinuclear antibody with a speckled pattern, cholestatic hepatitis with sinusoidal obstruction morphology, and CD8 dominant lobular infiltrates. The present study suggests HAA may result from cytotoxic T-cell-mediated sinusoidal endothelial and hepatocytic injury.
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Aplastic anaemia in Christchurch Hospital 1979-89.
Baker, B W; Smith, M P; Abbott, G D; Beard, M E; Spearing, R L; Heaton, D C
1991-11-13
We have reviewed the records of all patients referred to our departments with aplastic anaemia during the 11 years from 1979 to 1989. Of the 22 patients identified, 19 fulfilled the standard criteria for severe aplastic anaemia. There were 11 females and 11 males. Their mean age was 35 (range 2-85 years). Five cases followed exposure to drugs known to cause aplastic anaemia and one had a recent history of viral hepatitis. A variety of treatments were used. Four patients received an allogeneic bone marrow transplant (BMT) from matched sibling donors and two of these were alive and well 65 and 120 months post BMT. Antithymocyte globulin (ATG) treatment has been followed by lasting complete remission in two of the six patients treated and a partial response was seen in one other patient. Cyclosporin therapy was associated with unmaintained complete remission in one of the three patients given this drug after ATG had failed. The remaining 13 patients received only supportive care with or without androgens and six (46%) had early recovery of bone marrow function with lasting complete remission. These patients illustrate some of the therapeutic options available for aplastic anaemia.
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... the number of these blood cell types. Aplastic anemia can be caused by: Use of certain drugs or exposure to toxic chemicals (such as benzene) Exposure to radiation or chemotherapy Autoimmune disorders Pregnancy Viruses Sometimes, the cause is unknown. In this ...
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Idiopathic aplastic anemia: diagnosis and classification.
Dolberg, Osnat Jarchowsky; Levy, Yair
2014-01-01
Aplastic anemia (AA) is a disease characterized by pancytopenia and hypoplastic bone marrow caused by the decrease of hematopoietic stem cells. The pathogenesis of AA is complex and involves an abnormal hematopoietic microenvironment, hematopoietic stem cell/progenitor cell deficiencies and immunity disorders. Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. Herein, we will update the main issues concern AA according to our literature review. Copyright © 2014 Elsevier B.V. All rights reserved.
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Abuljadayel, Ilham Saleh; Mohanty, Dipika; Suri, Rajendar K.
2012-01-01
Background & objectives: Aplastic anaemia is a life threatening rare bone marrow failure disorder. The underlying haematopoietic cellular deficit leads to haemorrhage, infection and severe anaemia. The treatment of choice for this haematological condition is allogeneic bone marrow transplantation from fully matched HLA sibling. Though this procedure is curative in the majority of young patients with aplastic anaemia, extending this benefit to older patients or those lacking a family donor remains a major challenge. Herein, the safety and efficacy of infusing autologous retrodifferentiated haematopoietic stem cells (RHSC) into four patients with aplastic anaemia without the use of any pre- or post-conditioning regimen including immunosuppression is described. Methods: Un-mobilized, mononuclear cells were harvested from four patients with acquired aplastic anaemia by aphaeresis. Mononuclear cells of patients were cultured with purified monoclonal antibody against the monomorphic regions of the beta chain of MHC class II antigens (Clone CR3/43) for 3 h, to obtain autologous RHSC. Autologous RHSC were washed and infused into the four patients without the use of any pre- or post-conditioning regimen. Thereafter, the efficacy (engraftment) of autologous RHSC was assessed in these patients. Results: Following single infusion of the autologous RHSC, two of the four patients with aplastic anaemia become transfusion independent for more than seven years. Karyotyping and G-banding analysis prior and post-procedure in all patients remained the same. Interpretation & conclusions: The findings of this pilot study demonstrated the functional utility of reprogrammed fully differentiated adult cells into pluripotent stem cells with extensive repopulation potentials in a human setting and without any pre- or post-conditioning regimen, including immunosuppression. This autologous approach of stem cell creation may broaden the curative potentials of stem cell therapy to a wider population of patients with aplastic anaemia, including many patients suffering from other haematological and non-haematological disorders. PMID:22825605
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Xie, X T; He, W; Shi, W; Zhou, X X; Qiao, X H
2016-04-01
This study was designed to compare the effects of the anti-human T lymphocyte globulin (Fresenius, ATG-F)and rabbit anti-human thymocyte immunoglobulin (Genzyme, R-ATG)in the treatment of childhood aplastic anemia (AA) and their effects. A total of 59 children with aplastic anemia were analyzed in the present study, including 34 cases of severe aplastic anemia (SAA), 12 cases of very severe aplastic anemia (VSAA) and 13 cases of transfusion-dependent non-severe aplastic anemia (NSAA). While receiving immunosuppressive therapy (IST), 30 and 29 patients, with long-term oral supplement with cyclosporin A (CSA), androgen and Chinese traditional medicines, were treated with ATG-F and R-ATG, respectively. When it was necessary, some supportive cares such as component transfusion and infection control were also employed. Absolute counts of peripheral blood lymphocyte (ALC) at various time points were dynamically detected after ATG therapy. According to the International Aplastic Anemia Treatment and Effect standards. There were no statistically significant differences in the overall response rate (67%(20/30)vs. 69%(20/29), χ(2)=0.036, P=0.676) and the survival rate (87%(26/30)vs. 83%(24/29), χ(2)=0.173, P=0.676) between the ATG-F and R-ATG groups. There were significant and long-term ALC decrease after ATG therapy, the rate of ALC decrease in ATG-F and R-ATG group, the ALC only recovered to 47.8% (ATG-F group) and 47.4% (R-ATG group) of the pre-treatment level respectively. ATG-F 5 mg/(kg·d) and R-ATG 3.75 mg/(kg·d)could achieve similar effects in the treatment of childhood AA, through similar significant clearance of T cells. Therefore, all of these suggest that ATG-F and R-ATG might serve as the drugs of front-line choice for IST in childhood AA patients who do not have an available human leukocyte antigen identical related donor.
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Abuljadayel, Ilham Saleh; Mohanty, Dipika; Suri, Rajendar K
2012-06-01
Aplastic anaemia is a life threatening rare bone marrow failure disorder. The underlying haematopoietic cellular deficit leads to haemorrhage, infection and severe anaemia. The treatment of choice for this haematological condition is allogeneic bone marrow transplantation from fully matched HLA sibling. Though this procedure is curative in the majority of young patients with aplastic anaemia, extending this benefit to older patients or those lacking a family donor remains a major challenge. Herein, the safety and efficacy of infusing autologous retrodifferentiated haematopoietic stem cells (RHSC) into four patients with aplastic anaemia without the use of any pre- or post-conditioning regimen including immunosuppression is described. Un-mobilized, mononuclear cells were harvested from four patients with acquired aplastic anaemia by aphaeresis. Mononuclear cells of patients were cultured with purified monoclonal antibody against the monomorphic regions of the beta chain of MHC class II antigens (Clone CR3/43) for 3 h, to obtain autologous RHSC. Autologous RHSC were washed and infused into the four patients without the use of any pre- or post-conditioning regimen. Thereafter, the efficacy (engraftment) of autologous RHSC was assessed in these patients. Following single infusion of the autologous RHSC, two of the four patients with aplastic anaemia become transfusion independent for more than seven years. Karyotyping and G-banding analysis prior and post-procedure in all patients remained the same. The findings of this pilot study demonstrated the functional utility of reprogrammed fully differentiated adult cells into pluripotent stem cells with extensive repopulation potentials in a human setting and without any pre- or post-conditioning regimen, including immunosuppression. This autologous approach of stem cell creation may broaden the curative potentials of stem cell therapy to a wider population of patients with aplastic anaemia, including many patients suffering from other haematological and non-haematological disorders.
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Aplastic anemia during pregnancy: a review of obstetric and anesthetic considerations
Riveros-Perez, Efrain; Hermesch, Amy C; Barbour, Linda A; Hawkins, Joy L
2018-01-01
Aplastic anemia is a hematologic condition occasionally presenting during pregnancy. This pathological process is associated with significant maternal and neonatal morbidity and mortality. Obstetric and anesthetic management is challenging, and treatment requires a coordinated effort by an interdisciplinary team, in order to provide safe care to these patients. In this review, we describe the current state of the literature as it applies to the complexity of aplastic anemia in pregnancy, focusing on pathophysiologic aspects of the disease in pregnancy, as well as relevant obstetric and anesthetic considerations necessary to treat this challenging problem. A multidisciplinary-team approach to the management of aplastic anemia in pregnancy is necessary to coordinate prenatal care, optimize maternofetal outcomes, and plan peripartum interventions. Conservative transfusion management is critical to prevent alloimmunization. Although a safe threshold-platelet count for neuraxial anesthesia has not been established, selection of anesthetic technique must be evaluated on a case-to-case basis. PMID:29535558
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Yoshida, Nao; Kobayashi, Ryoji; Yabe, Hiromasa; Kosaka, Yoshiyuki; Yagasaki, Hiroshi; Watanabe, Ken-Ichiro; Kudo, Kazuko; Morimoto, Akira; Ohga, Shouichi; Muramatsu, Hideki; Takahashi, Yoshiyuki; Kato, Koji; Suzuki, Ritsuro; Ohara, Akira; Kojima, Seiji
2014-12-01
The current treatment approach for severe aplastic anemia in children is based on studies performed in the 1980s, and updated evidence is required. We retrospectively compared the outcomes of children with acquired severe aplastic anemia who received immunosuppressive therapy within prospective trials conducted by the Japanese Childhood Aplastic Anemia Study Group or who underwent bone marrow transplantation from an HLA-matched family donor registered in the Japanese Society for Hematopoietic Cell Transplantation Registry. Between 1992 and 2009, 599 children (younger than 17 years) with severe aplastic anemia received a bone marrow transplant from an HLA-matched family donor (n=213) or immunosuppressive therapy (n=386) as first-line treatment. While the overall survival did not differ between patients treated with immunosuppressive therapy or bone marrow transplantation [88% (95% confidence interval: 86-90) versus 92% (90-94)], failure-free survival was significantly inferior in patients receiving immunosuppressive therapy than in those undergoing bone marrow transplantation [56% (54-59) versus 87% (85-90); P<0.0001]. There was no significant improvement in outcomes over the two time periods (1992-1999 versus 2000-2009). In multivariate analysis, age <10 years was identified as a favorable factor for overall survival (P=0.007), and choice of first-line immunosuppressive therapy was the only unfavorable factor for failure-free survival (P<0.0001). These support the current algorithm for treatment decisions, which recommends bone marrow transplantation when an HLA-matched family donor is available in pediatric severe aplastic anemia. Copyright© Ferrata Storti Foundation.
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Yoshida, Nao; Kobayashi, Ryoji; Yabe, Hiromasa; Kosaka, Yoshiyuki; Yagasaki, Hiroshi; Watanabe, Ken-ichiro; Kudo, Kazuko; Morimoto, Akira; Ohga, Shouichi; Muramatsu, Hideki; Takahashi, Yoshiyuki; Kato, Koji; Suzuki, Ritsuro; Ohara, Akira; Kojima, Seiji
2014-01-01
The current treatment approach for severe aplastic anemia in children is based on studies performed in the 1980s, and updated evidence is required. We retrospectively compared the outcomes of children with acquired severe aplastic anemia who received immunosuppressive therapy within prospective trials conducted by the Japanese Childhood Aplastic Anemia Study Group or who underwent bone marrow transplantation from an HLA-matched family donor registered in the Japanese Society for Hematopoietic Cell Transplantation Registry. Between 1992 and 2009, 599 children (younger than 17 years) with severe aplastic anemia received a bone marrow transplant from an HLA-matched family donor (n=213) or immunosuppressive therapy (n=386) as first-line treatment. While the overall survival did not differ between patients treated with immunosuppressive therapy or bone marrow transplantation [88% (95% confidence interval: 86–90) versus 92% (90–94)], failure-free survival was significantly inferior in patients receiving immunosuppressive therapy than in those undergoing bone marrow transplantation [56% (54–59) versus 87% (85–90); P<0.0001]. There was no significant improvement in outcomes over the two time periods (1992–1999 versus 2000–2009). In multivariate analysis, age <10 years was identified as a favorable factor for overall survival (P=0.007), and choice of first-line immunosuppressive therapy was the only unfavorable factor for failure-free survival (P<0.0001). These support the current algorithm for treatment decisions, which recommends bone marrow transplantation when an HLA-matched family donor is available in pediatric severe aplastic anemia. PMID:25193958
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Chakrabarti, Sudipta; Goswami, Bidyut Krishna; Paul, Prabir Chandra; Sarkar, Supriya; Mondal, Srikrishna; Das, Shikha
2013-10-01
There is a scarcity of clinical data in the field of aplastic anaemia from rural India. Present study was conducted in North Bengal Medical College to find out the clinicohaematological profile and the possible aetiological factors in patients with aplastic anaemia. The study population (n = 83) included 21 children with male to female ratio 4.93:1. Weakness was present in all cases and pallor was present in 74.70% cases. Fever, bleeding episodes and localised infection were found in 55.42%, 48.19% and 27.71% cases respectively. There was no difference in clinical manifestations between children and adult except occurrence of fever (p = 0.0365). We identified possible aetiological factors in 32.53% cases as relevant drug intake in 10.84%, exposure to chemicals in 13.25% and hepatitis in 8.43% cases. We found low mean haemoglobin (3.81 +/- 1.71 g/dl), leucocyte and platelet (3.05 +/- 1.3 and 37.30 +/- 35 x 10(3)/cmm), neutrophil (30.28 +/- 21.76%) and high lymphocyte (67.27 +/- 22.50%) in peripheral blood. At the time of presentation, aplastic anaemia was moderate in 59.04% cases, severe in 48.19% cases and mild in 4.82% cases. We estimated the prevalence of aplastic anaemia in 4 districts of West Bengal including tea garden areas was 2.98/million populations per year. It was concluded that aplastic anaemia is a major non-malignant haematological disease in this part of India, and an increasing use of chemicals in agricultural and tea garden areas might be the responsible factor. Larger population based study is suggested.
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Detection of Parvovirus B19 Infection in Thalasemic Patients in Isfahan Province, Iran
Nikoozad, Razieh; Mahzounieh, Mohammad Reza; Ghorani, Mohammad Reza
2015-01-01
Background: Parvovirus B19, a member of the Erythrovirus genus of Parvoviridae family, causes various clinical illnesses including infectious erythema, arthropathy, hydrops fetalis or congenital anemia, and transient aplastic crises. The B19 virus can be transmitted through respiratory secretions, blood products, and blood transfusion. Objectives: The aim of this study was to detect the B19 virus in thalassemia patients in Isfahan, Iran. Patients and Methods: The prevalence of parvovirus B19 infection was compared between thalassemia major patients and healthy subjects. Plasma samples were collected from 30 thalassemia patients from Isfahan, Iran. Thirty patients without any blood complications were considered as the control group. After DNA extraction from the plasma samples, polymerase chain reaction was performed for parvovirus B19 detection. Results: The parvovirus B19-specific nucleotide sequence was detected in 6 patients (20%). None of the samples obtained from the 30 control subjects tested positive for B19. Conclusions: In this study B19-Parvovirus infection were detected in patients with hematologic disorders in comparison with control subjects. Screening of patients with a high risk of parvovirus B19 infection can considerably reduce the incidence and prevalence of B19 infection. PMID:26855745
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Detection of Parvovirus B19 Infection in Thalasemic Patients in Isfahan Province, Iran.
Nikoozad, Razieh; Mahzounieh, Mohammad Reza; Ghorani, Mohammad Reza
2015-11-01
Parvovirus B19, a member of the Erythrovirus genus of Parvoviridae family, causes various clinical illnesses including infectious erythema, arthropathy, hydrops fetalis or congenital anemia, and transient aplastic crises. The B19 virus can be transmitted through respiratory secretions, blood products, and blood transfusion. The aim of this study was to detect the B19 virus in thalassemia patients in Isfahan, Iran. The prevalence of parvovirus B19 infection was compared between thalassemia major patients and healthy subjects. Plasma samples were collected from 30 thalassemia patients from Isfahan, Iran. Thirty patients without any blood complications were considered as the control group. After DNA extraction from the plasma samples, polymerase chain reaction was performed for parvovirus B19 detection. The parvovirus B19-specific nucleotide sequence was detected in 6 patients (20%). None of the samples obtained from the 30 control subjects tested positive for B19. In this study B19-Parvovirus infection were detected in patients with hematologic disorders in comparison with control subjects. Screening of patients with a high risk of parvovirus B19 infection can considerably reduce the incidence and prevalence of B19 infection.
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2012-11-07
Acute Myeloid Leukemia; Myelodysplasia; Acute Lymphoblastic Leukemia; Chronic Lymphocytic Leukemia; Follicular Lymphoma; Multiple Myeloma; NHL; Myeloproliferative Diseases; Chronic Myeloid Leukemia; Renal Cell Carcinoma; Aplastic Anemia
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Meta-analysis of treatment with rabbit and horse antithymocyte globulin for aplastic anemia.
Hayakawa, Jin; Kanda, Junya; Akahoshi, Yu; Harada, Naonori; Kameda, Kazuaki; Ugai, Tomotaka; Wada, Hidenori; Ishihara, Yuko; Kawamura, Koji; Sakamoto, Kana; Ashizawa, Masahiro; Sato, Miki; Terasako-Saito, Kiriko; Kimura, Shun-Ichi; Kikuchi, Misato; Yamazaki, Rie; Kako, Shinichi; Kanda, Yoshinobu
2017-05-01
Aplastic anemia patients who received rabbit antithymocyte globulin exhibited response and survival rates inferior to those who received horse antithymocyte globulin in several studies. Therefore, we conducted a meta-analysis to compare rabbit and horse antithymocyte globulin as immunosuppressive therapy for aplastic anemia. We searched online databases for studies that compared antithymocyte globulin regimens as first-line treatment for aplastic anemia, including both randomized and non-randomized controlled trials. The early mortality rate at 3 months and overall response rate at 6 months were evaluated. Thirteen studies were included in the analysis. The risk ratio (RR) of early mortality for rabbit vs. horse antithymocyte globulin was 1.33 [95% confidence interval (CI) 0.69-2.57; P = 0.39], with significant heterogeneity. A sensitivity analysis suggested higher early mortality rate in patients who received rabbit antithymocyte globulin. The overall response rate was significantly higher in patients who received horse antithymocyte globulin (RR 1.27; 95% CI 1.05-1.54; P = 0.015). In conclusion, in aplastic anemia patients treated with ATG, early mortality rate was not significantly different in patients receiving horse or rabbit ATG, although a sensitivity analysis showed higher early mortality in the rabbit ATG group. Horse ATG was associated with significantly higher response rate than rabbit ATG.
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Invasive Infection with Fusarium chlamydosporum in a Patient with Aplastic Anemia
Segal, Brahm H.; Walsh, Thomas J.; Liu, Johnson M.; Wilson, Jon D.; Kwon-Chung, Kyung J.
1998-01-01
We report the first case of invasive disease caused by Fusarium chlamydosporum. The patient had aplastic anemia with prolonged neutropenia and was treated with immunosuppressive therapy. While she was receiving empirical amphotericin B, a dark crusted lesion developed on her nasal turbinate. Histologic analysis revealed invasive hyaline hyphae and some darkly pigmented structures that resembled conidia of dematiaceous molds. Only after the mold was grown in culture were characteristic colonial morphology, phialides, conidia, and chlamydospores evident, thus permitting the identification of F. chlamydosporum. This case illustrates the ever-increasing spectrum of pathogenic Fusarium spp. in immunocompromised patients and emphasizes the potential pitfalls in histologic diagnosis, which may have important treatment implications. PMID:9620419
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An Animal Model of Chronic Aplastic Bone Marrow Failure Following Pesticide Exposure in Mice
Chatterjee, Sumanta; Chaklader, Malay; Basak, Pratima; Das, Prosun; Das, Madhurima; Pereira, Jacintha Archana; Dutta, Ranjan Kumar; Chaudhuri, Samaresh; Law, Sujata
2010-01-01
The wide use of pesticides for agriculture, domestic and industrial purposes and evaluation of their subsequent effect is of major concern for public health. Human exposure to these contaminants especially bone marrow with its rapidly renewing cell population is one of the most sensitive tissues to these toxic agents represents a risk for the immune system leading to the onset of different pathologies. In this experimental protocol we have developed a mouse model of pesticide(s) induced hypoplastic/aplastic marrow failure to study quantitative changes in the bone marrow hematopoietic stem cell (BMHSC) population through flowcytometric analysis, defects in the stromal microenvironment through short term adherent cell colony (STACC) forming assay and immune functional capacity of the bone marrow derived cells through cell mediated immune (CMI) parameter study. A time course dependent analysis for consecutive 90 days were performed to monitor the associated changes in the marrow’s physiology after 30th, 60th and 90th days of chronic pesticide exposure. The peripheral blood showed maximum lowering of the blood cell count after 90 days which actually reflected the bone marrow scenario. Severe depression of BMHSC population, immune profile of the bone marrow derived cells and reduction of adherent cell colonies pointed towards an essentially empty and hypoplastic marrow condition that resembled the disease aplastic anemia. The changes were accompanied by splenomegaly and splenic erythroid hyperplasia. In conclusion, this animal model allowed us a better understanding of clinico-biological findings of the disease aplastic anemia following toxic exposure to the pesticide(s) used for agricultural and industrial purposes. PMID:24855541
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Heyrman, Bert; De Becker, Ann; Verheyden, Sonja; Demanet, Christian
2017-03-02
The aim of this case report is to draw attention on possible false human leucocyte antigen (HLA) genotyping in acquired aplastic anaemia prior to allogeneic haematopoietic stem cell transplantation. In acquired aplastic anaemia loss of heterozygosity (LOH) of chromosome 6p is known to occur in around 12%. We report false HLA genotyping results due to LOH and a coinciding steep rise in neutrophils following filgrastim stimulation in a patient with very severe aplastic anaemia. At diagnosis we obtained heterozygous results on peripheral blood. Failing to reach a partial response at 6 months with immune-suppressive therapy we repeated HLA genotyping, obtaining homozygous results. Repeated testing confirmed loss of HLA genotype heterozygosity. HLA genotyping on cells obtained by a buccal swab confirmed the previous HLA heterozygosity. A second course of filgrastim at the time of homozygous HLA genotyping resulted in a steep rise in neutrophils. Stopping filgrastim resulted in an equally steep drop. 2017 BMJ Publishing Group Ltd.
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Dasouki, Majed J; Rafi, Syed K; Olm-Shipman, Adam J; Wilson, Nathan R; Abhyankar, Sunil; Ganter, Brigitte; Furness, L Mike; Fang, Jianwen; Calado, Rodrigo T; Saadi, Irfan
2013-11-14
We recently identified 2 siblings afflicted with idiopathic, autosomal recessive aplastic anemia. Whole-exome sequencing identified a novel homozygous missense mutation in thrombopoietin (THPO, c.112C>T) in both affected siblings. This mutation encodes an arginine to cysteine substitution at residue 38 or residue 17 excluding the 21-amino acid signal peptide of THPO receptor binding domain (RBD). THPO has 4 conserved cysteines in its RBD that form 2 disulfide bonds. Our in silico modeling predicts that introduction of a fifth cysteine may disrupt normal disulfide bonding to cause poor receptor binding. In functional assays, the mutant-THPO-containing media shows two- to threefold reduced ability to sustain UT7-TPO cells, which require THPO for proliferation. Both parents and a sibling with heterozygous R17C change have reduced platelet counts, whereas a sibling with wild-type sequence has normal platelet count. Thus, the R17C partial loss-of-function allele results in aplastic anemia in the homozygous state and mild thrombocytopenia in the heterozygous state in our family. Together with the recent identification of THPO receptor (MPL) mutations and the effects of THPO agonists in aplastic anemia, our results have clinical implications in the diagnosis and treatment of patients with aplastic anemia and highlight a role for the THPO-MPL pathway in hematopoiesis in vivo.
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Chatterjee, Sumanta; Basak, Pratima; Das, Prosun; Das, Madhurima; Pereira, Jacintha Archana; Dutta, Ranjan Kumar; Chaklader, Malay; Chaudhuri, Samaresh; Law, Sujata
2010-01-01
Self-renewing Hematopoietic Stem Cells (HSCs) are responsible for reconstitution of all blood cell lineages. Sca-1 is the “stem cell antigen” marker used to identify the primitive murine HSC population, the expression of which decreases upon differentiation to other mature cell types. Sca-1+ HSCs maintain the bone marrow stem cell pool throughout the life. Aplastic anemia is a disease considered to involve primary stem cell deficiency and is characterized by severe pancytopenia and a decline in healthy blood cell generation system. Studies conducted in our laboratory revealed that the primitive Sca-1+ BM-HSCs (bone marrow hematopoietic stem cell) are significantly affected in experimental Aplastic animals pretreated with chemotherapeutic drugs (Busulfan and Cyclophosphamide) and there is increased Caspase-3 activity with consecutive high Annexin-V positivity leading to premature apoptosis in the bone marrow hematopoietic stem cell population in Aplastic condition. The Sca-1bright, that is, “more primitive” BM-HSC population was more affected than the “less primitive” BM-HSC Sca-1dim population. The decreased cell population and the receptor expression were directly associated with an empty and deranged marrow microenvironment, which is evident from scanning electron microscopy (SEM). The above experimental evidences hint toward the manipulation of receptor expression for the benefit of cytotherapy by primitive stem cell population in Aplastic anemia cases. PMID:21048851
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Clinical management of aplastic anemia
DeZern, Amy E; Brodsky, Robert A
2011-01-01
Acquired aplastic anemia is a potentially fatal bone marrow failure disorder that is characterized by pancytopenia and a hypocellular bone marrow. Hematopoietic stem-cell transplantation or bone marrow transplantation (BMT) is the treatment of choice for young patients who have a matched sibling donor. Immunosuppression with either anti-thymocyte globulin and cyclosporine or high-dose cyclophosphamide is an effective therapy for patients who are not suitable BMT candidates owing to age or lack of a suitable donor. Results of BMT from unrelated and mismatched donors are improving, but presently this treatment option is best reserved for those patients who do not respond, relapse or develop secondary clonal disorders following immunosuppressive therapy. Efforts are currently underway to both improve immunosuppressive regimens and to expand the application of BMT. PMID:21495931
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ERIC Educational Resources Information Center
Birbirso, Dereje Tadesse
2014-01-01
Since 2000, Ethiopia has been working to come out of social crises, modernise itself and achieve the Millennium Development Goals. Although provided with billions of dollars by the West and their international agents, little has been changed and the crises seem never to abate, especially in the educational system. This study, thus, critically…
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Onishi, Yasushi; Mori, Takehiko; Kako, Shinichi; Koh, Hideo; Uchida, Naoyuki; Kondo, Tadakazu; Kobayashi, Takeshi; Yabe, Hiromasa; Miyamoto, Toshihiro; Kato, Koji; Suzuki, Ritsuro; Nakao, Shinji; Yamazaki, Hirohito
2017-12-01
Graft failure (GF) is the most critical life-threatening complication of allogeneic hematopoietic stem cell transplantation (HSCT) for aplastic anemia, for which a second transplantation is the only effective treatment. Optimal procedures have not been established for the second transplantation in this setting, however. Here we retrospectively analyzed the outcomes of 22 patients with aplastic anemia, age ≥16 years, who underwent umbilical cord blood transplantation for GF after the first HSCT using the registry database of the Japan Society for Hematopoietic Cell Transplantation. The median age of patients was 36 years (range, 16 to 72 years), and the median time from the first to the second transplant was 77 days (range, 29 to 1061 days). The cumulative incidence of neutrophil engraftment at day 60 post-transplantation was 45.5% (95% confidence interval [CI], 23.6% to 65.0%). With a median follow-up of 50 months, the 4-year overall survival (OS) was 38.5% (95% CI, 18.4% to 58.5%). Mycofenolate mofetil-based graft-versus-host disease prophylaxis demonstrated greater neutrophil recovery than prophylaxis with calcineurin inhibitor alone or methotrexate-based prophylaxis (66.7% versus 37.5%; P = .04). The use of such conditioning regimens as fludarabine + melphalan or cyclophosphamide + low-dose total body irradiation was associated with better engraftment (58.3% versus 30%; P = .05) and better 4-year OS (55.6% versus 20%; P = .05) than other regimens. Although further investigation is needed, umbilical cord blood could be an effective and promising option for stem cell source for urgent second transplantation in patients with aplastic anemia who develop GF after the first HSCT. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.
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Periodontal management of a patient with severe aplastic anemia: a case report.
Agnihotri, Rupali; Bhat, Khandige Mahalinga; Bhat, Giliyar Subraya; Pandurang, Pratibha
2009-01-01
This article describes the periodontal management of a patient with severe aplastic anemia. A 51-year-old female patient was hospitalized with aplastic anemia and was referred for the management of her cyclosporine-induced gingival enlargement and bleeding from her gums. She also complained of pain in the palatal area and the mandibular anterior region. The patient was managed successfully with supra- and subgingival instrumentation, local drug delivery (LDD), electrosurgery, repeated oral hygiene instruction, and constant supervision. The treatment resulted in dramatic improvement in the patient's oral health and quality of life. This report highlights the importance of nonsurgical therapy followed by electrosurgery for the management of residual gingival enlargement.
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Najean, Y; Pecking, A; Le Mercier, N; Zittoun, R; Chaussade, R
1978-06-10
The sex, age, occupation and residence of 251 patients with aplastic anaemia in adults have been analyzed, and compared to those of the french population. The following observations have been done: excess of males at each age beyond the 20th year; excessive frequency after the 50th year; excessive frequency in the rural population and the inhabitants of villages and little towns; but no excess in the industrial workers. These observations lead to minimize the aetiological responsibility of industrial toxics in aplastic anaemia; at the reverse they invite to look for a possible responsibility of one or several agents used in the rural world.
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Balfour, Margaret E; Tanner, Kathleen; Jurica, Paul J; Rhoads, Richard; Carson, Chris A
2016-01-01
Crisis and emergency psychiatric services are an integral part of the healthcare system, yet there are no standardized measures for programs providing these services. We developed the Crisis Reliability Indicators Supporting Emergency Services (CRISES) framework to create measures that inform internal performance improvement initiatives and allow comparison across programs. The framework consists of two components-the CRISES domains (timely, safe, accessible, least-restrictive, effective, consumer/family centered, and partnership) and the measures supporting each domain. The CRISES framework provides a foundation for development of standardized measures for the crisis field. This will become increasingly important as pay-for-performance initiatives expand with healthcare reform.
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Demir, Esen; Cigerci Günaydın, Nurşen; Karadaş, Nihal; Gülen, Figen; Tanac, Remziye; Yılmaz, Deniz
2015-03-01
Horse antithymocyte globulin (h-ATG) (ATGAM(®) ) is the first choice of treatment in very severe patients with aplastic anemia who do not have any HLA matched sibling donor. h-ATG is a heterologous serum that may cause anaphylaxis. Alternative treatment strategies must be planned in case of hypersensitivity. Desensitization must be considered in patients without an alternative treatment of choice. We aimed to present the h-ATG desensitization protocol and consider its effectiveness in patients with aplastic anemia who are hypersensitized with h-ATG and do not have an alternative treatment of choice. Skin prick tests were performed with non-diluted solution in eight very severe patients with aplastic anemia who are followed up in Ege University Children's Hospital. Although skin prick test was found negative in these eight patients, different dilution h-ATG intradermal tests were performed and found positive in all patients. h-ATG desensitization program was started to these hypersensitized patients. Desensitization program was started to six male and two female very severe patients with aplastic anemia whose ages were between seven and 19 yr (median: 12.9 yr). All of the patients completed the desensitization program. While local reaction was seen in two patients, systemic reaction was seen in one patient and late reaction was seen in one patient during and after desensitization program. A successful desensitization program with h-ATG in children with aplastic anemia is presented. Even though there is not an exposure before to such high allergy potential heterologous serum, skin tests should be performed and desensitization must be started to patients who are hypersensitized to h-ATG. As the expected effectiveness of the treatment is so much, the desensitization protocol can be carried out safely and effectively with trained stuff although allergic reactions can be seen. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
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Collier, Anderson B; Coon, Lea M; Monteleone, Philip; Umaru, Samuel; Swanson, Kenneth C; Hoyer, James D; Oliveira, Jennifer L
2016-01-01
Hemoglobin (Hb) variants may be associated with low oxygen saturation and exacerbated episodes of anemia from common stressors such as viral infections. These attributes frequently cause increased clinical concern and unnecessary and expensive testing if not considered early in the evaluation of the patient. Some clinically significant Hb variants result in a normal Hb electrophoresis result, which can be method-dependent. Herein we describe a patient with low oxygen saturation and a history of hemolytic anemia who was subsequently found to carry a novel, unstable β-globin variant that we have named Hb Allentown [β137(H15)Val→Trp (GTG>TGG) HBB: c.412_413delinsTG, p.Val138Trp] for the place of identification of the variant. Hb Allentown is formed by a rare double nucleotide substitution within the same codon. Additionally, positive identification of rare Hb variants characterized by a single method is discouraged, as the Hb variant was misclassified as Hb S-South End or β6(A3)Glu→Val;β132(H10)Lys→Asn (HBB: c.[20A > T;399A > C]) by the initial laboratory.
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Invasive Sino-Orbital Mycosis in an Aplastic Anemia Patient Caused by Neosartorya laciniosa
Malejczyk, Kathy; Sigler, Lynne; Gibas, Connie Fe C.
2013-01-01
We report the first case of Neosartorya laciniosa invasive sinusitis involving the orbit in an immunocompromised male with aplastic anemia. Treatment included surgical debridement with enucleation of the eye and combination voriconazole and micafungin therapy followed by voriconazole alone. The fungus was identified using sequencing of partial benA and calmodulin genes. PMID:23345294
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Steiner, Laurie A; Van Hoff, Jack; Kutlar, Ferdane; Gallagher, Patrick G
2007-01-01
This report describes a Hispanic girl who presented in aplastic crisis due to parvovirus infection. She was subsequently found to have a chronic hemolytic anemia secondary to an unstable hemoglobinopathy. Genetic testing revealed that the girl and two symptomatic family members had Hb Evans [alpha62(E11)Val-->Met, GTG-->ATG (alpha2)], an unstable hemoglobin (Hb) variant due to a mutation in the alpha2-globin chain. Hb Evans has been described only once previously, in a Caucasian kindred. Literature review indicates aplastic crisis is a rare initial presentation of unstable hemoglobinopathies and that these disorders are infrequent but important causes of hemolytic anemia in Hispanic patients.
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COMPARISON OF BIOMARKERS IN WORKERS EXPOSED TO 2,4,6-TRINITROTOLUENE
2,4,6-Trinitrotoluene (TNT) is an important occupational and environmental pollutant. In TNT-exposed humans, the notable toxic manifestations have included aplastic anemia, toxic hepatitis, cataracts, hepatomegaly, and liver cancer. Therefore, we developed methods to biomonitor w...
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Keel, Siobán B.; Scott, Angela; Sanchez-Bonilla, Marilyn; Ho, Phoenix A.; Gulsuner, Suleyman; Pritchard, Colin C.; Abkowitz, Janis L.; King, Mary-Claire; Walsh, Tom; Shimamura, Akiko
2016-01-01
The clinical and histopathological distinctions between inherited versus acquired bone marrow failure and myelodysplastic syndromes are challenging. The identification of inherited bone marrow failure/myelodysplastic syndromes is critical to inform appropriate clinical management. To investigate whether a subset of pediatric and young adults undergoing transplant for aplastic anemia or myelodysplastic syndrome have germline mutations in bone marrow failure/myelodysplastic syndrome genes, we performed a targeted genetic screen of samples obtained between 1990–2012 from children and young adults with aplastic anemia or myelodysplastic syndrome transplanted at the Fred Hutchinson Cancer Research Center. Mutations in inherited bone marrow failure/myelodysplastic syndrome genes were found in 5.1% (5/98) of aplastic anemia patients and 13.6% (15/110) of myelodysplastic syndrome patients. While the majority of mutations were constitutional, a RUNX1 mutation present in the peripheral blood at a 51% variant allele fraction was confirmed to be somatically acquired in one myelodysplastic syndrome patient. This highlights the importance of distinguishing germline versus somatic mutations by sequencing DNA from a second tissue or from parents. Pathological mutations were present in DKC1, MPL, and TP53 among the aplastic anemia cohort, and in FANCA, GATA2, MPL, RTEL1, RUNX1, SBDS, TERT, TINF2, and TP53 among the myelodysplastic syndrome cohort. Family history or physical examination failed to reliably predict the presence of germline mutations. This study shows that while any single specific bone marrow failure/myelodysplastic syndrome genetic disorder is rare, screening for these disorders in aggregate identifies a significant subset of patients with inherited bone marrow failure/myelodysplastic syndrome. PMID:27418648
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[Hematopoietic stem-cell transplantation in aplastic anemia].
Hernández-Rivera, E Gabriela
2005-01-01
Severe aplastic anemia is a rare syndrome characterized by bone marrow failure with cytopenias and hypocellular bone marrow biopsy (usually 10-15%), without blasts or myelodysplasia. The first choice treatment for these patients is allogeneic bone marrow transplantation from a sibling matched for HLA-A, HLA-B and HLA-DR. Unfortunately only 30% of patients have an HLA-matched sibling (a 25% chance per sibling). The alternative treatment for severe aplastic anemia for the rest of the patients (70%) is immunosuppression with antithymocyte globuline and cyclosporine. The evolution of bone marrow transplantation since 1970's has been positive in terms of survival and transplant success (initial overall survival 43% vs. 90% lately, and graft rejection of 29% vs. 4%). The favorable outcome of bone marrow transplantation for severe or very severe aplastic anemia is due to: the use of conditioning with antithymocyte globuline and cyclophosphamide, the use of graft-vs.-host disease prophylaxis with short curse methotrexate and cyclosporine and the use of filtrated and irradiated blood products. For those patients without an HLA-matched related donor the first treatment to use is the immunosuppression with antithymocyte globuline and cyclosporine. Another option emerged in the late 80's is the unrelated bone marrow transplantation, with survival hardly half of the HLA-identical related bone marrow transplants. In our country, the first allogeneic bone marrow transplant was done in the Instituto Nacional de Ciencias Médicas y Nutrición, Salvador Zubirán, in a patient with aplastic anemia, making possible to perform this procedure safely in our country.
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Acquired Aplastic Anemia in Children
Hartung, Helge D.; Olson, Timothy S.; Bessler, Monica
2013-01-01
SYNOPSIS This article provides a practice-based and concise review of the etiology, diagnosis, and management of acquired aplastic anemia in children. Bone marrow transplantation, immunosuppressive therapy, and supportive care are discussed in detail. The aim is to provide the clinician with a better understanding of the disease and to offer guidelines for the management of children with this uncommon yet serious disorder. PMID:24237973
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[Aplastic crisis in sickle cell anemia induced by parvovírus B19
Borsato, M L; Bruniera, P; Cusato, M P; Spewien, K E; Durigon, E L; Toporovski, J
2000-01-01
PURPOSE: Transient aplastic crisis is reported in an eight-month old child with sickle cell anemia and acute B19 parvovirus infection. This fact is uncommon in this age. PATIENT AND METHODS: The authors review the literature and describe a clinical case of an eight-month old child with sickle cell anemia presented with profound anemia and reticulocytopenia. His peripheral blood was analyzed for parvovirus B19 using the polymerase chain reaction (PCR), and for anti B19 immunoglobulin Ig M, and Ig G by enzyme-linked immunosorbent assay (ELISA). RESULTS: An eight-month old child with sickle cell anemia was admitted to the hospital with fever and profound anemia (HB = 3.8g/ dl) and reticulocytopenia (2%). A diagnosis of aplastic crisis was established. The results indicate that Ig M and PCR were positive and Ig G negative. The patient needed erytrocyte transfusion, and was discharged on hospital day 4. CONCLUSIONS: The clinical and laboratory features indicate that human parvovirus B19 was the etiologic agent of an aplastic crisis in an eight-month old child. According to the international literature this event is uncommon for this age; in addition, this is the first time it appears in the Brazilian literature.
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Eltrombopag: a review of its use in patients with severe aplastic anaemia.
McCormack, Paul L
2015-04-01
Eltrombopag (Promacta®) is an orally active thrombopoietin receptor agonist recently approved in the US for the treatment of patients with severe aplastic anaemia who have had an insufficient response to immunosuppressive therapy. This article reviews the efficacy and tolerability of eltrombopag in this indication and overviews its pharmacological properties. Eltrombopag does not compete with thrombopoietin and binds to a different site on the receptor, producing additive effects. It stimulates haematopoietic stem cells and promotes haematopoietic recovery in patients with aplastic bone marrow. Eltrombopag increased platelet counts and can also increase red blood cell and neutrophil counts. In patients with severe aplastic anaemia refractory to prior immunosuppressive therapy, oral eltrombopag at dosages ≤150 mg once daily for 12-16 weeks produced a haematological response in at least one cell lineage in 40 % of patients. Trilineage responses were achieved in nearly one-half of the responders during extended treatment. In robust responders, stable haematological counts were maintained after eltrombopag discontinuation. Eltrombopag was generally well tolerated, with increased liver transaminases as the only dose-limiting toxicity. Clonal cytogenetic abnormalities were observed in 19 % of patients and dysplasia in 5 % of patients.
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Caring and Coping. Contemporary Issues: Facing Family Crises.
ERIC Educational Resources Information Center
Mosatche, Harriet S.
This booklet focuses on three particularly prevalent family crises: divorce, the death of a parent, and poverty. These crises profoundly affect millions of children in their personal, academic, and social development. The information and activities in this booklet are provided to enable Girl Scout leaders to increase their efforts both in helping…
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Gérvas, Juan; Meneu, Ricard
2010-12-01
The perception, acceptability and management of risks are social construction. Consequently, in managing public health crises, the gap between facts, beliefs and feelings tests the responsiveness of official institutions to health alarms that can be objective, potential, or imaginary. On balance, a strong point of the Spanish experience of health crises is the presence of clinicians and public health officers working in an organization capable of responding adequately, although the quasi-federal Spanish political structure has both advantages and disadvantages. Weaknesses include the low profile given to public health and a management structure that relies too heavily on partitocracy. The management of these crises could be improved by transferring greater scope to health professionals in decisions about crisis identification and management (with transparency) and limiting bureaucratic inertia. For some, health crises involve visibility or business opportunities (not always legitimate). Therefore, the perception of crisis will increasingly rest less in the hands of experts and more in those of groups interested in spreading these crises or in providing solutions. While progress is needed to develop participation in strategies to respond to emerging crises, even more essential is the involvement of all healthcare levels in their preparation and dissemination. Copyright © 2010 SESPAS. Published by Elsevier Espana. All rights reserved.
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Matsue, K.; Niki, T.; Shiobara, S.
1990-01-01
We describe the clinical course of a 16 year old girl with aplastic anemia who was treated by syngeneic bone marrow transplantation. Engraftment was not obtained by simple infusion of bone marrow without immunosuppression. The patient received a high-dose cyclophosphamide and thoracoabdominal irradiation, followed by second marrow transplantation from the same donor. Incomplete but significant hematologic recovery was observed; however, marrow failure recurred 5 months after transplantation. Since donor and recipient pairs were genotypically identical, graft failure could not be attributed to immunological reactivity of recipient cells to donor non-HLA antigens. This case report implies that graft failure in somemore » cases of aplastic anemia might be mediated by inhibitory cells resistant to cyclophosphamide and irradiation.« less
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Han, J R; Yuan, S W; Ren, Q F
1990-06-01
Fresh fetal liver obtained from 3- to 6-month fetus was prepared. Fetal liver cell suspension (FLC) or fetal liver cell-free suspension (FLCF) were then transfused into two groups of patient of aplastic anemia. 15 of 21 patients of aplastic anemia treated with FLC showed reconstitution of haemopoietic function or improvement of peripheral blood pictures, while 27 of 30 patients treated with FLCF showed reconstitution or improvement. It is verified that there is a stimulating factor for CFU-CM, BFU-E, and CFU-E and also a immunologic stimulant for improving the nonspecific immunologic function of the organism as shown by clinical analysis and experimental study. It is obvious that the therapeutic effect of FLCF is much better than that of the FLC.
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Strengthening the evidence base for health programming in humanitarian crises.
Ager, A; Burnham, G; Checchi, F; Gayer, M; Grais, R F; Henkens, M; Massaquoi, M B F; Nandy, R; Navarro-Colorado, C; Spiegel, P
2014-09-12
Given the growing scale and complexity of responses to humanitarian crises, it is important to develop a stronger evidence base for health interventions in such contexts. Humanitarian crises present unique challenges to rigorous and effective research, but there are substantial opportunities for scientific advance. Studies need to focus where the translation of evidence from noncrisis scenarios is not viable and on ethical ways of determining what happens in the absence of an intervention. Robust methodologies suited to crisis settings have to be developed and used to assess interventions with potential for delivery at scale. Strengthening research capacity in the low- to middle-income countries that are vulnerable to crises is also crucial. Copyright © 2014, American Association for the Advancement of Science.
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Ohara, A; Kojima, S; Hamajima, N; Tsuchida, M; Imashuku, S; Ohta, S; Sasaki, H; Okamura, J; Sugita, K; Kigasawa, H; Kiriyama, Y; Akatsuka, J; Tsukimoto, I
1997-08-01
The improved outcome of acquired aplastic anemia (AA) has revealed later complications, such as myelodysplastic syndrome (MDS) and acute myelogenous leukemia (AML). We retrospectively analyzed 167 children with severe acquired AA. Eleven of 50 children treated with cyclosporin (CSA) and recombinant human granulocyte colony-stimulating factor (rhG-CSF) developed MDS/AML; 8 of these were within 36 months of the diagnosis of AA, much earlier than previous reports. Six of the 11 children received rhG-CSF exceeding 10 microg/kg/d, and 9 received rhG-CSF therapy for over 1 year. Ten children showed monosomy 7 at diagnosis of MDS. All of the 11 children were administered both CSA and rhG-CSF. There was no development of MDS/AML among 41 children treated with either CSA or rhG-CSF or among 48 children who underwent bone marrow transplantation. A well-controlled clinical trial is warranted to determine whether therapeutic modalities affect the development of MDS/AML in children with severe acquired AA.
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Scheinberg, Phillip; Marte, Michael; Nunez, Olga; Young, Neal S.
2010-01-01
Background Clones of glycosylphosphatidylinositol-anchor protein-deficient cells are characteristic in paroxysmal nocturnal hemoglobinuria and are present in about 40–50% of patients with severe aplastic anemia. Flow cytometry has allowed for sensitive and precise measurement of glycosylphosphatidylinositol-anchor protein-deficient red blood cells and neutrophils in severe aplastic anemia. Design and Methods We conducted a retrospective analysis of paroxysmal nocturnal hemoglobinuria clones measured by flow cytometry in 207 consecutive severe aplastic anemia patients who received immunosuppressive therapy with a horse anti-thymocyte globulin plus cyclosporine regimen from 2000 to 2008. Results The presence of a glycosylphosphatidylinositol-anchor protein-deficient clone was detected in 83 (40%) patients pre-treatment, and the median clone size was 9.7% (interquartile range 3.5–29). In patients without a detectable clone pre-treatment, the appearance of a clone after immunosuppressive therapy was infrequent, and in most with a clone pre-treatment, clone size often decreased after immunosuppressive therapy. However, in 30 patients, an increase in clone size was observed after immunosuppressive therapy. The majority of patients with a paroxysmal nocturnal hemoglobinuria clone detected after immunosuppressive therapy did not have an elevated lactate dehydrogenase, nor did they experience hemolysis or thrombosis, and they did not require specific interventions with anticoagulation and/or eculizumab. Of the 7 patients who did require therapy for clinical paroxysmal nocturnal hemoglobinuria symptoms and signs, all had an elevated lactate dehydrogenase and a clone size greater than 50%. In all, 18 (8.6%) patients had a clone greater than 50% at any given time of sampling. Conclusions The presence of a paroxysmal nocturnal hemoglobinuria clone in severe aplastic anemia is associated with low morbidity and mortality, and specific measures to address clinical paroxysmal nocturnal hemoglobinuria are seldom required. PMID:20595102
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Narita, Atsushi; Muramatsu, Hideki; Sekiya, Yuko; Okuno, Yusuke; Sakaguchi, Hirotoshi; Nishio, Nobuhiro; Yoshida, Nao; Wang, Xinan; Xu, Yinyan; Kawashima, Nozomu; Doisaki, Sayoko; Hama, Asahito; Takahashi, Yoshiyuki; Kudo, Kazuko; Moritake, Hiroshi; Kobayashi, Masao; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji
2015-01-01
Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length were identified as predictive biomarkers of immunosuppressive therapy responsiveness in aplastic anemia. We enrolled 113 aplastic anemia patients (63 boys and 50 girls) in this study to evaluate their response to immunosuppressive therapy. The paroxysmal nocturnal hemoglobinuria populations and telomere length were detected by flow cytometry. Forty-seven patients (42%) carried a minor paroxysmal nocturnal hemoglobinuria population. The median telomere length of aplastic anemia patients was −0.99 standard deviation (SD) (range −4.01–+3.01 SD). Overall, 60 patients (53%) responded to immunosuppressive therapy after six months. Multivariate logistic regression analysis identified the absence of a paroxysmal nocturnal hemoglobinuria population and a shorter telomere length as independent unfavorable predictors of immunosuppressive therapy response at six months. The cohort was stratified into a group of poor prognosis (paroxysmal nocturnal hemoglobinuria negative and shorter telomere length; 37 patients) and good prognosis (paroxysmal nocturnal hemoglobinuria positive and/or longer telomere length; 76 patients), respectively. The response rates of the poor prognosis and good prognosis groups at six months were 19% and 70%, respectively (P<0.001). The combined absence of a minor paroxysmal nocturnal hemoglobinuria population and a short telomere length is an efficient predictor of poor immunosuppressive therapy response, which should be considered while deciding treatment options: immunosuppressive therapy or first-line hematopoietic stem cell transplantation. The trial was registered in www.umin.ac.jp with number UMIN000017972. PMID:26315930
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Devillier, Raynier; Dalle, Jean-Hugues; Kulasekararaj, Austin; D'aveni, Maud; Clément, Laurence; Chybicka, Alicja; Vigouroux, Stéphane; Chevallier, Patrice; Koh, Mickey; Bertrand, Yves; Michallet, Mauricette; Zecca, Marco; Yakoub-Agha, Ibrahim; Cahn, Jean-Yves; Ljungman, Per; Bernard, Marc; Loiseau, Pascale; Dubois, Valérie; Maury, Sébastien; Socié, Gérard; Dufour, Carlo; Peffault de Latour, Regis
2016-07-01
Unrelated allogeneic transplantation for severe aplastic anemia is a treatment option after immunosuppressive treatment failure in the absence of a matched sibling donor. Age, delay between disease diagnosis and transplantation, and HLA matching are the key factors in transplantation decisions, but their combined impact on patient outcomes remains unclear. Using the French Society of Bone Marrow Transplantation and Cell Therapies registry, we analyzed all consecutive patients (n=139) who underwent a first allogeneic transplantation for idiopathic severe aplastic anemia from an unrelated donor between 2000 and 2012. In an adjusted multivariate model, age over 30 years (Hazard Ratio=2.39; P=0.011), time from diagnosis to transplantation over 12 months (Hazard Ratio=2.18; P=0.027) and the use of a 9/10 mismatched unrelated donor (Hazard Ratio=2.14; P=0.036) were independent risk factors that significantly worsened overall survival. Accordingly, we built a predictive score using these three parameters, considering patients at low (zero or one risk factors, n=94) or high (two or three risk factors, n=45) risk. High-risk patients had significantly shorter survival (Hazard Ratio=3.04; P<0.001). The score was then confirmed on an independent cohort from the European Group for Blood and Marrow Transplantation database of 296 patients, with shorter survival in patients with at least 2 risk factors (Hazard Ratio=2.13; P=0.005) In conclusion, a simple score using age, transplantation timing and HLA matching would appear useful to help physicians in the daily care of patients with severe aplastic anemia. Copyright© Ferrata Storti Foundation.
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Narita, Atsushi; Muramatsu, Hideki; Sekiya, Yuko; Okuno, Yusuke; Sakaguchi, Hirotoshi; Nishio, Nobuhiro; Yoshida, Nao; Wang, Xinan; Xu, Yinyan; Kawashima, Nozomu; Doisaki, Sayoko; Hama, Asahito; Takahashi, Yoshiyuki; Kudo, Kazuko; Moritake, Hiroshi; Kobayashi, Masao; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji
2015-12-01
Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length were identified as predictive biomarkers of immunosuppressive therapy responsiveness in aplastic anemia. We enrolled 113 aplastic anemia patients (63 boys and 50 girls) in this study to evaluate their response to immunosuppressive therapy. The paroxysmal nocturnal hemoglobinuria populations and telomere length were detected by flow cytometry. Forty-seven patients (42%) carried a minor paroxysmal nocturnal hemoglobinuria population. The median telomere length of aplastic anemia patients was -0.99 standard deviation (SD) (range -4.01-+3.01 SD). Overall, 60 patients (53%) responded to immunosuppressive therapy after six months. Multivariate logistic regression analysis identified the absence of a paroxysmal nocturnal hemoglobinuria population and a shorter telomere length as independent unfavorable predictors of immunosuppressive therapy response at six months. The cohort was stratified into a group of poor prognosis (paroxysmal nocturnal hemoglobinuria negative and shorter telomere length; 37 patients) and good prognosis (paroxysmal nocturnal hemoglobinuria positive and/or longer telomere length; 76 patients), respectively. The response rates of the poor prognosis and good prognosis groups at six months were 19% and 70%, respectively (P<0.001). The combined absence of a minor paroxysmal nocturnal hemoglobinuria population and a short telomere length is an efficient predictor of poor immunosuppressive therapy response, which should be considered while deciding treatment options: immunosuppressive therapy or first-line hematopoietic stem cell transplantation. The trial was registered in www.umin.ac.jp with number UMIN000017972. Copyright© Ferrata Storti Foundation.
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Vaht, Krista; Göransson, Magnus; Carlson, Kristina; Isaksson, Cecilia; Lenhoff, Stig; Sandstedt, Anna; Uggla, Bertil; Winiarski, Jacek; Ljungman, Per; Brune, Mats; Andersson, Per-Ola
2017-01-01
A plastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70–80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000–2011. Patients were included via the National Patient Registry, and diagnosed according to the Camitta criteria. In total, 257 confirmed cases were identified, with an overall incidence of 2.35 (95% CI: 2.06–2.64) cases per million inhabitants per year. Median age was 60 years (range: 2–92), and median follow up was 76 (0–193) months. Primary treatments included immunosuppressive therapy (63%), allogenic stem cell transplantation (10%), or single-agent cyclosporine/no specific therapy (27%). The 5-year survival was 90.7% in patients aged 0–18 years, 90.5% in patients aged 19–39 years, 70.7% in patients aged 40–59 years, and 38.1% in patients aged ≥60 years. Multivariate analysis showed that age (both 40–59 and ≥60 age groups), very severe aplastic anemia and single-agent cyclosporine/no specific therapy were independent risk factors for inferior survival. In conclusion, younger aplastic anemia patients experience a very good long-term survival, while that of patients ≥60 years in particular remains poor. Apparently, the challenge today is to improve the management of older aplastic anemia patients, and prospective studies to address this medical need are warranted. PMID:28751565
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Vaht, Krista; Göransson, Magnus; Carlson, Kristina; Isaksson, Cecilia; Lenhoff, Stig; Sandstedt, Anna; Uggla, Bertil; Winiarski, Jacek; Ljungman, Per; Brune, Mats; Andersson, Per-Ola
2017-10-01
A plastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70-80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000-2011. Patients were included via the National Patient Registry, and diagnosed according to the Camitta criteria. In total, 257 confirmed cases were identified, with an overall incidence of 2.35 (95% CI: 2.06-2.64) cases per million inhabitants per year. Median age was 60 years (range: 2-92), and median follow up was 76 (0-193) months. Primary treatments included immunosuppressive therapy (63%), allogenic stem cell transplantation (10%), or single-agent cyclosporine/no specific therapy (27%). The 5-year survival was 90.7% in patients aged 0-18 years, 90.5% in patients aged 19-39 years, 70.7% in patients aged 40-59 years, and 38.1% in patients aged ≥60 years. Multivariate analysis showed that age (both 40-59 and ≥60 age groups), very severe aplastic anemia and single-agent cyclosporine/no specific therapy were independent risk factors for inferior survival. In conclusion, younger aplastic anemia patients experience a very good long-term survival, while that of patients ≥60 years in particular remains poor. Apparently, the challenge today is to improve the management of older aplastic anemia patients, and prospective studies to address this medical need are warranted. Copyright© 2017 Ferrata Storti Foundation.
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Lüders, Florian; Engelbertz, Christiane; Meyborg, Matthias; Freisinger, Eva; Malyar, Nasser M; Zeller, Thomas; Reinecke, Holger
2016-06-01
Evident data about the additive effect of "the fifth cardiovascular risk factor" (anemia) and peripheral arterial disease (PAD) focused on morbidity and outcome of patients with PAD are currently still missing. A total of 41,882 PAD patients were included. Of these, 5566 (13.3%) suffered from anemia. Patients with anemia were older (P<0.001), suffered more often from chronic kidney disease (P<0.001), coronary artery disease (P<0.001), and more severe PAD (P<0.001). However, they received significantly less endovascular revascularizations (P<0.001), had higher amputation rates (acute anemia: 3.7-fold, P<0.001; nutritional, aplastic, and anemia in chronic disease: 2.9-fold, P<0.001), higher in-hospital mortality rates (acute anemia: 6.4-fold, P<0.001; nutritional, aplastic, and anemia in chronic disease: 4.6-fold; P<0.001), had significantly higher in-hospital complications (P<0.001) compared to those without anemia. During a follow-up time up to 4years (until Dec. 31st, 2012, median 775days, 25th-75th percentiles 469-1120days) nutritional, aplastic, and anemia in chronic disease and acute anemia were high significant predictors of long-term mortality and amputation (each P<0.001). Lengths of hospital stay and reimbursement costs were higher (nutritional, aplastic, and anemia in chronic disease: 2-fold higher (P<0.001), acute anemia: 3-fold higher (P<0.001)) than in patients without anemia. This study illustrates from a large, comprehensive database the association of acute, nutritional, aplastic, and anemia in chronic disease on morbidity, in-hospital treatment and complications, short- and long term outcome, and costs of patients with PAD. Copyright © 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
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Eltrombopag in aplastic anaemia
Desmond, Ronan; Townsley, Danielle M.; Dunbar, Cynthia; Young, Neal S.
2014-01-01
The treatment of aplastic anemia is currently with immunosuppressive therapy (IST) with antithymocyte globulin and cyclosporine, to which two thirds of patients respond. However, a significant proportion of these responders relapse and many have persistent cytopenias. The management of these patients is challenging. Modifications to this standard approach using alternative immunosuppressive agents or adding hematopoietic cytokines such as G-CSF and erythropoietin have not improved outcome. A recent trial has shown that eltrombopag, a thrombopoeitin mimetic, is efficacious in the treatment of patients with severe aplastic anemia (SAA) refractory to IST. There is evidence that this drug works by directly stimulating marrow stem and progenitor cells thereby promoting hematopoietic recovery in patients with bone marrow failure. Several trials are ongoing in our institution using this very promising drug in combination therapy in the upfront treatment of SAA, in IST refractory SAA and in moderate disease. PMID:25578417
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NASA Astrophysics Data System (ADS)
Popov, Dmitri
Key Words: Aplastic Anemia (AA), Pluripotential Stem Cells (PSC) Introduction: Aplastic Anemia (AA) is a disorder of the pluripotential stem cells involve a decrease in the number of cells of myeloid, erythroid and megakaryotic lineage [Segel et al. 2000 ]. The etiology of AA include idiopathic cases and secondary aplastic anemia after exposure to drugs, toxins, chemicals, viral infections, lympho-proliferative diseases, radiation, genetic causes, myelodisplastic syndromes and hypoplastic anemias, thymomas, lymphomas. [Brodskyet al. 2005.,Modan et al. 1975., Szklo et al. 1975]. Hematopoietic Acute Radiation Syndrome (or Bone marrow syndrome, or Radiation-Acquired Aplastic Anemia) is the acute toxic syndrome which usually occurs with a dose of irradiation between 0.7 and 10 Gy (70- 1000 rads), depending on the species irradiated. [Waselenko et al., 2004]. The etiology of bone morrow damage from high-level radiation exposure results depends on the radiosensitivity of certain bone marrow cell lines. [Waselenko et al. 2004] Aplastic anemia after radiation exposure is a clinical syndrome that results from a marked disorder of bone marrow blood cell production. [Waselenko et al. 2004] Radiation hematotoxicity is mediated via genotoxic and other specific toxic mechanisms, leading to aplasia, cell apoptosis or necrosis, initiation via genetic mechanisms of clonal disorders, in cases such as the acute radiation-acquired form of AA. AA results from radiation injury to pluripotential and multipotential stem cells in the bone marrow. The clinical signs displayed in reticulocytopenia, anemia, granulocytopenia, monocytopenia, and thrombocytopenia. The number of marrow CD34+ cells (multipotential hematopoietic progenitors) and their derivative colony-forming unit{granulocyte-macrophage (CFU-GM) and burst forming unit {erythroid (BFU{E) are reduced markedly in patients with AA. [Guinan 2011, Brodski et al. 2005, Beutler et al.,2000] Cells expressing CD34 (CD34+ cell) are normally found in the umbilical cord and bone marrow as hematopoietic cells, a subset of mesenchymal stem cells, endothelial progenitor cells, endothelial cells of blood vessels, etc. [Beutler et al. 2000 ] Potential mechanisms responsible for radiation-acquired marrow cell failure include direct toxicity , direct damage of hematopoietic multipotential cells or cellular or humoral immune suppression of the marrow multipotential cells. [ Beutler et al. 2000] Methods: These studies were conducted at several different research institutions and laboratories listed as follows: Kazan All-Union Scientific Research Veterinary, Biotechnology Centre of Russian Academy of Science (North Osetia), Institute Belarussian Scientific and Research Institute for Radiobiology in Gomel, the St. Petersburg Veterinary Institute, the Advanced Medical Technology and Systems Inc., Ontario, Canada. The studies were approved by the Animal Care and Use Committee for ethical animal research equivalent, at each institution. A critically important volume of purified Radiation Toxins (RT) was isolated from larger mammalian irradiated animals. Subsequently the RT were characterized chemically and biologically. The experimental design of later studies compared relative toxicity, potential for development of acute radiation hematopoietic syndrome, and potential cloning disorder of multipotential hematopoietic progenitors and their derivative and lethality after intravenous or intramuscular injections of SRD containing Hematopoietic Radiation Toxins. These experiments have employed a wide variety of experimental animals. The animals were irradiated in RUM-17, Puma, and Panorama devices. The dose varied from 0.7Gy to 100Gy. The methods of immune depletion, immuno-lympho plasmasabsorption, as well as direct extraction, were used to refine and purify the specific Radiation Toxins from the central lymph of animals with Hematopoietic forms of Radiation Toxins. Experiments include administration of Hematopoietic Radiation Toxins (SRD-4) to radiation naive animals in doses 0.1 mg/kg; 0,5 mg/kg; 1 mg/kg; 2 mg/kg; 3 mg/kg up to 30 mg/kg. Results: After I/V or I/M administration of Hematotoxic Radiation Toxins to radiation -naive animals the induction of specific clinical signs was observed- including thrombocytopenia, lymphocytosis followed by lymphocytopenia, granulocytopenia , aplastic anemia, and the clinical manifestations- ecchymosis, hemorrhage and coagulopathy. These observed clinical signs mimic the acute/hematopoietic acute radiation syndrome. Conclusions: Administration of Hematopoietic Radiation Toxins (SRD-4) to radiation naive animals in doses 0.1 mg/kg;0,5 mg/kg; 1 mg/kg; 2 mg/kg; 3 mg/kg up to 30 mg/kg produced specific toxic reactions with the development of signs and symptoms consistent with the hematological form of Acute Radiation Syndromes. Administration of high doses of Hematopoietic Radiation Toxins developed a clinical picture identical to severe Acute Radiation Exposure Syndrome and induces Toxic Multiple Organ Failure (TMOF) and Toxic Multiple Organ Involvement (TMOI) {i.e. pneumonitis, renal failure, renal hypo-perfusion, acute tubular necrosis, hepatic failure, etc.} essentially as which occurs as an acute consequence of radiation toxemia. Aplastic anemia is an important clinical and pathological process which develops after animals receive high doses of both radiation and administered radiation toxins.
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Estimating Groundwater Development area in Jianan Plain using Standardized Groundwater Index
NASA Astrophysics Data System (ADS)
Yu, Chang Hsiang; Haw, Lee Cheng
2017-04-01
Taiwan has been facing severe water crises in recent years owing to the effects of extreme weather conditions. Changes in precipitation patterns have also made the drought phenomenon increasingly prominent, which has indirectly affected groundwater recharge. Hence, in the present study, long-term monitoring data were collected from the study area of the Jianan plain. The standardized groundwater index (SGI) and was then used to analyse the region's drought characteristics. To analyse the groundwater level by using SGI, making SGI180 groundwater level be the medium water crises, and SGI360 groundwater level be the extreme water crises. Through the different water crises signal in SGI180 and SGI360, we divide groundwater in Jianan plain into two sections. Thereby the water crises indicators establishing groundwater level standard line in Jianan Plain, then using the groundwater level standard line to find the study area where could be groundwater development area in Jianan plain. Taking into account relatively more water scarcity in dry season, so the study screen out another emergency backup groundwater development area, but the long-term groundwater development area is still as a priority development area. After finding suitable locations, groundwater modeling systems(GMS) software is used to simulate our sites to evaluate development volume. Finally, the result of study will help the government to grasp the water shortage situation immediately and solve the problem of water resources deployment.
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Strategies for Crises Prevention/Intervention.
ERIC Educational Resources Information Center
Webber, Glen R.
Crises intervention and prevention techniques developed by the Echo Glen Children's Center school in Washington are listed here, along with a description of climate improvement and problem-solving methods developed by the school's staff. The students in this special residential school are emotionally disturbed or socially maladjusted delinquent…
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Congenital aplastic-hypoplastic lumbar pedicle in infants and young children
DOE Office of Scientific and Technical Information (OSTI.GOV)
Yousefzadeh, D.K.; El-Khoury, G.Y.; Lupetin, A.R.
1982-01-01
Nine cases of congenital aplastic-hypoplastic lumbar pedicle (mean age 27 months) are described. Their data are compared to those of 18 other reported cases (mean age 24.7 years) and the following conclusions are made: (1) Almost exclusively, the pedicular defect in infants and young children is due to developmental anomaly rather than destruction by malignancy or infectious processes. (2) This anomaly, we think, is more common than it is believed to be. (3) Unlike adults, infants and young children rarely develop hypertrophy and/or sclerosis of the contralateral pedicle. (4) Detection of pedicular anomaly is more than satisfying a radiographic curiositymore » and may lead to discovery of other coexisting anomalies. (5) Ultrasonic screening of the patients with congenital pedicular defects may detect the associated genitourinary anomalies, if present, and justify further studies in a selected group of patients.« less
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Redox Regulation in Bone Marrow Failure
2012-06-01
Fanconi anemia mutation for hematopoietic senescence. J Cell Sci, 2007. 120(Pt 9): p. 1572-83. 2. Aylon, Y. and M. Oren, Living with p53, dying of p53...aplastic anemia patients with a p38 MAPK inhibitor can restore defective hematopoietic activity, suggesting the critical role of p38 in bone marrow...hematopoietic stem cells, and eventually leading to bone marrow failure [7, 8] [9] [10]. On the other hand, treating aplastic anemia patients with a p38
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Kekre, Natasha; Zhang, Ying; Zhang, Mei-Jie; Carreras, Jeanette; Ahmed, Parvez; Anderlini, Paolo; Atta, Elias Hallack; Ayas, Mouhab; Boelens, Jaap Jan; Bonfim, Carmem; Deeg, H Joachim; Kapoor, Neena; Lee, Jong-Wook; Nakamura, Ryotaro; Pulsipher, Michael A; Eapen, Mary; Antin, Joseph H
2017-07-01
For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin results in more profound immunosuppression. We analyzed 833 severe aplastic anemia transplants between 2008 and 2013 using human leukocyte antigen (HLA)-matched siblings (n=546) or unrelated donors (n=287) who received antithymocyte globulin as part of their conditioning regimen and bone marrow graft. There were no differences in hematopoietic recovery by type of antithymocyte globulin. Among recipients of HLA-matched sibling transplants, day 100 incidence of acute (17% versus 6%, P <0.001) and chronic (20% versus 9%, P <0.001) graft- versus -host disease were higher with horse compared to rabbit antithymocyte globulin. There were no differences in 3-year overall survival, 87% and 92%, P =0.76, respectively. Among recipients of unrelated donor transplants, acute graft- versus -host disease was also higher with horse compared to rabbit antithymocyte globulin (42% versus 23%, P <0.001) but not chronic graft- versus -host disease (38% versus 32%, P =0.35). Survival was lower with horse antithymocyte globulin after unrelated donor transplantation, 75% versus 83%, P =0.02. These data support the use of rabbit antithymocyte globulin for bone marrow transplant conditioning for severe aplastic anemia. Copyright© 2017 Ferrata Storti Foundation.
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Case of subcutaneous abscess caused by Nocardia farcinica in an aplastic anemia patient.
Yokota, Sho; Kawabe, Keitaro; Yamada, Hideki; Nunomura, Maki
2010-01-01
We report a case of subcutaneous abscess caused by Nocardia farcinica in a 44-year-old man, who had been treated with systemic prednisolone and cyclosporin for aplastic anemia. He had been affected by aplastic anemia for 8 years, and was previously treated with antithymocyte globulin. The effect was insufficient, and platelet and erythrocyte transfusion was required. Bone marrow transplantation was not adopted due to a psychological problem. He had also been treated with prednisolone and cyclosporin for 3 years. Without apparent cause, swelling and pain of left upper extremity developed in April, 2008. There was no abnormality in cutaneous macroscopic findings, such as a wound or a sting. He was administered antibiotics, but they were ineffective and effusion were excreted into the skin. After hospitalization, he was treated by incision and drainage and antibiotics were started. The pathogen was identified as Nocardia farcinica by its biochemical characters. There was no dissemination to other organs like lung or brain and he recovered completely with a treatment of antibiotics for 1 year. We studied the epidemiological and clinical characteristics of Nocardia infections reported in Japan from 2000 to 2008 and identified 92 cases using the medical article search engine Ichushi-Web (Japan Medical Abstract Society). The results indicate that the most important risk factor in systemic nocardiosis ia an immunosuppressive agent such as prednisolone, cyclosporine, or azathioprine. We believe that we should take the possibility of Nocardia infection into consideration in a compromised host.
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Babushok, Daria V.; Grignon, Anne‐Laure; Li, Yimei; Atienza, Jamie; Xie, Hongbo M.; Lam, Ho‐Sun; Hartung, Helge; Bessler, Monica
2016-01-01
Hepatitis‐associated aplastic anemia (HAA) is a variant of acquired aplastic anemia (AA) in which immune‐mediated bone marrow failure (BMF) develops following an acute episode of seronegative hepatitis. Dyskeratosis congenita (DC) is an inherited BMF syndrome characterized by the presence of short telomeres, mucocutaneous abnormalities, and cancer predisposition. While both conditions may cause BMF and hepatic impairment, therapeutic approaches are distinct, making it imperative to establish the correct diagnosis. In clinical practice, lymphocyte telomere lengths (TL) are used as a first‐line screen to rule out inherited telomeropathies before initiating treatment for AA. To evaluate the reliability of TL in the HAA population, we performed a retrospective analysis of TL in 10 consecutively enrolled HAA patients compared to 19 patients with idiopathic AA (IAA). HAA patients had significantly shorter telomeres than IAA patients (P = 0.009), including four patients with TL at or below the 1st percentile for age‐matched controls. HAA patients had no clinical features of DC and did not carry disease‐causing mutations in known genes associated with inherited telomere disorders. Instead, short TLs were significantly correlated with severe lymphopenia and skewed lymphocyte subsets, features characteristic of HAA. Our results indicate the importance of caution in the interpretation of TL measurements in HAA, because, in this patient population, short telomeres have limited specificity. Am. J. Hematol. 91:243–247, 2016. © 2015 The Authors. American Journal of Hematology Published by Wiley Periodicals, Inc. PMID:26615915
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Varicella-Like Cutaneous Toxoplasmosis in a Patient with Aplastic Anemia
Zimmermann, Stefan; Hadaschik, Eva; Dalpke, Alexander; Hassel, Jessica C.; Ajzenberg, Daniel; Tenner-Racz, Klara; Lehners, Nicola; Kapaun, Annette
2013-01-01
A 60-year-old patient with aplastic anemia presented with vesicular varicella-like skin lesions on her face, arms, legs, back, and abdomen. However, diagnosis for herpetic infection was negative. Findings of a skin biopsy led to a tentative histologic diagnosis of toxoplasmosis, and infection with Toxoplasma gondii was confirmed by immunohistochemistry and PCR. Cutaneous toxoplasmosis is a rare finding in immunocompromised patients and might mimic other infectious diseases, and vesicular lesions associated with toxoplasmosis have not been reported previously. PMID:23390283
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2006-07-01
abnormalities in constitutional aplastic anemia . NEng JMed 274:8–14. Callen E, Samper E, Ramirez MJ, Creus A, Marcos R, Ortega JJ, Olive T, Badell I, Blasco... anemia , ‘‘idiopathic’’ aplastic anemia , and Fanconi anemia heterozy- gotes. Am J Med Genet 15:211–223. Chaganti RSK, Houldsworth J. 1991. Fanconi anemia : A...allows us to apply the GPA assay, regardless of genotype, for diagnosis of the cancer-prone diseases ataxia telangiectasia (9), Fanconi anemia (8
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Zhang, Xuejie; Lin, Shengyun; Yang, Yan; Rong, Liucheng; He, Guangsheng; He, Hailong; Xue, Yao; Fang, Yongjun; Wang, Yaping
2017-01-01
Cytokines IL-2 and IL-8 both participate in immune regulation. However, the relationship between polymorphisms in these two cytokines and the risk of acquired aplastic anemia (acquired AA) has not been explored. We selected five SNPs including rs11575812, rs2069772 and rs2069762 of IL-2, rs2227306 and rs2227543 of IL-8. SNaPshot genotyping was used to test the genotypes of IL-2 and IL-8 polymorphisms in a population of 101 acquired AA patients and 165 healthy controls. The rs2069762 G allele appeared to be a protective mutation, but no significant differences were found in other four SNPs. We also found that rs2069762 had an impact on the transcriptional regulation. It could be assumed that the rs2069762 polymorphism might reduce the risk of acquired aplastic anemia, while the remaining four SNPs might not contribute to susceptibility to acquired AA in a Chinese population. © 2017 The Author(s)Published by S. Karger AG, Basel.
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Dufour, Carlo; Pillon, Marta; Sociè, Gerard; Rovò, Alicia; Carraro, Elisa; Bacigalupo, Andrea; Oneto, Rosi; Passweg, Jakob; Risitano, Antonio; Tichelli, Andrè; Peffault de Latour, Regis; Schrezenmeier, Hubert; Hocshmann, Britta; Peters, Christina; Kulasekararaj, Austin; Van Biezen, Anja; Samarasinghe, Sujith; Hussein, Ayad Ahmed; Ayas, Mouhab; Aljurf, Mahmoud; Marsh, Judith
2015-05-01
This study analysed the outcome of 563 Aplastic Anaemia (AA) children aged 0-12 years reported to the Severe Aplastic Anaemia Working Party database of the European Society for Blood and Marrow Transplantation, according to treatment received. Overall survival (OS) after upfront human leucocyte antigen-matched family donor (MFD) haematopoietic stem cell transplantation (HSCT) or immunosuppressive treatment (IST) was 91% vs. 87% (P 0·18). Event-free survival (EFS) after upfront MFD HSCT or IST was 87% vs. 33% (P 0·001). Ninety-one of 167 patients (55%) failed front-line IST and underwent rescue HSCT. The OS of this rescue group was 83% compared with 91% for upfront MFD HSCT patients and 97% for those who did not fail IST up-front (P 0·017). Rejection was 2% for MFD HSCT and HSCT post-IST failure (P 0·73). Acute graft-versus-host disease (GVHD) grade II-IV was 8% in MFD graft vs. 25% for HSCT post-IST failure (P < 0·0001). Chronic GVHD was 6% in MFD HSCT vs. 20% in HSCT post-IST failure (P < 0·0001). MFD HSCT is an excellent therapy for children with AA. IST has a high failure rate, but remains a reasonable first-line choice if MFD HSCT is not available because high OS enables access to HSCT, which is a very good rescue option. © 2015 John Wiley & Sons Ltd.
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Commentary on Becoming a Daughter: Trauma is a powerful teacher.
Veach, Patricia McCarthy
2006-06-01
Personal life crises profoundly impact genetic counselor practice. In this commentary, themes from Matloff's (in press) article, Becoming a Daughter are highlighted and expanded upon. These themes include: personal impact of a life crisis, and professional impact vis a vis empathy countertransference, self-disclosure, nondirectiveness, and self-confidence. Strategies that help genetic counselors manage personal life crises within their clinical practice and also promote their professional development are emphasized, including normalization of life crises, self-reflection, boundary-setting, and use of peer supervision and consultation.
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Anal Canal Gas Gangrene in Aplastic Anaemia: Rare yet Lethal Entity - A Caveat.
Sudharsanan, Sundaramurthi; Vijayakumar, Chellappa; Manish, Kumar; Elamurugan, T P; Manwar, Ali S
2017-07-13
Gas gangrene is one of the most serious infections of traumatic and surgical wounds. The importance lies in the fact that, if not managed at the right time, the condition is fatal. Though perianal cellulitis and gangrene are commonly reported in immunocompromised patients, we report the case of a patient with gas gangrene involving only the anal canal extending to the rectum, a rare presentation to be reported in literature. An 18-year-old lady, a patient of aplastic anaemia - immunodeficiency, developed gas gangrene of the anal canal possibly due to faecal contamination of anal fissures. The patient was managed with surgical debridement and intravenous antibiotics. The clinical manifestations of gas gangrene are due to the liberation of toxins by Clostridium perfringens. The infection spreads rapidly and results in necrosis and devitalisation of tissues, unless intervened surgically. The clinical manifestations are more rapid and a high index of suspicion is needed for the diagnosis.
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Anal Canal Gas Gangrene in Aplastic Anaemia: Rare yet Lethal Entity - A Caveat
Sudharsanan, Sundaramurthi; Vijayakumar, Chellappa; Manish, Kumar; Elamurugan, TP
2017-01-01
Gas gangrene is one of the most serious infections of traumatic and surgical wounds. The importance lies in the fact that, if not managed at the right time, the condition is fatal. Though perianal cellulitis and gangrene are commonly reported in immunocompromised patients, we report the case of a patient with gas gangrene involving only the anal canal extending to the rectum, a rare presentation to be reported in literature. An 18-year-old lady, a patient of aplastic anaemia - immunodeficiency, developed gas gangrene of the anal canal possibly due to faecal contamination of anal fissures. The patient was managed with surgical debridement and intravenous antibiotics. The clinical manifestations of gas gangrene are due to the liberation of toxins by Clostridium perfringens. The infection spreads rapidly and results in necrosis and devitalisation of tissues, unless intervened surgically. The clinical manifestations are more rapid and a high index of suspicion is needed for the diagnosis. PMID:28936381
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Mavroudi, Irene; Papadaki, Helen A.
2012-01-01
Increasing interest on the field of autoimmune diseases has unveiled a plethora of genetic factors that predispose to these diseases. However, in immune-mediated bone marrow failure syndromes, such as acquired aplastic anemia and chronic idiopathic neutropenia, in which the pathophysiology results from a myelosuppressive bone marrow microenvironment mainly due to the presence of activated T lymphocytes, leading to the accelerated apoptotic death of the hematopoietic stem and progenitor cells, such genetic associations have been very limited. Various alleles and haplotypes of human leucocyte antigen (HLA) molecules have been implicated in the predisposition of developing the above diseases, as well as polymorphisms of inhibitory cytokines such as interferon-γ, tumor necrosis factor-α, and transforming growth factor-β1 along with polymorphisms on molecules of the immune system including the T-bet transcription factor and signal transducers and activators of transcription. In some cases, specific polymorphisms have been implicated in the outcome of treatment on those patients. PMID:22956967
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The complex pathophysiology of acquired aplastic anaemia
Zeng, Y; Katsanis, E
2015-01-01
Immune-mediated destruction of haematopoietic stem/progenitor cells (HSPCs) plays a central role in the pathophysiology of acquired aplastic anaemia (aAA). Dysregulated CD8+ cytotoxic T cells, CD4+ T cells including T helper type 1 (Th1), Th2, regulatory T cells and Th17 cells, natural killer (NK) cells and NK T cells, along with the abnormal production of cytokines including interferon (IFN)-γ, tumour necrosis factor (TNF)-α and transforming growth factor (TGF)-β, induce apoptosis of HSPCs, constituting a consistent and defining feature of severe aAA. Alterations in the polymorphisms of TGF-β, IFN-γ and TNF-α genes, as well as certain human leucocyte antigen (HLA) alleles, may account for the propensity to immune-mediated killing of HSPCs and/or ineffective haematopoiesis. Although the inciting autoantigens remain elusive, autoantibodies are often detected in the serum. In addition, recent studies provide genetic and molecular evidence that intrinsic and/or secondary deficits in HSPCs and bone marrow mesenchymal stem cells may underlie the development of bone marrow failure. PMID:25683099
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An Information-Processing Model of Crisis Management.
ERIC Educational Resources Information Center
Egelhoff, William G.; Sen, Falguni
1992-01-01
Develops a contingency model for managing a variety of corporate crises. Views crisis management as an information-processing situation and organizations that must cope with crisis as information-processing systems. Attempts to fit appropriate information-processing mechanisms to different categories of crises. (PRA)
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ERIC Educational Resources Information Center
Mitroff, Ian I.; Diamond, Michael A.; Alpaslan, Murat C.
2006-01-01
This article outlines a set of recommendations to college and university leaders and governing bodies on how to develop crisis-management systems to ensure that their institutions are as well prepared as possible for a wide range of crises. These recommendations are based, in part, on crisis-management programs developed for various business…
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Peinemann, Frank; Labeit, Alexander Michael
2014-07-15
Acquired severe aplastic anaemia is a rare and potentially fatal disease. The aim of this Cochrane review was to evaluate the effectiveness and adverse events of first-line allogeneic haematopoietic stem cell transplantation of human leucocyte antigen (HLA)-matched sibling donors compared with first-line immunosuppressive therapy. Specialised stem cell transplantations units in primary care hospitals. We included 302 participants with newly diagnosed acquired severe aplastic anaemia. The age ranged from early childhood to young adulthood. We excluded studies on participants with secondary aplastic anaemia. We included allogeneic haematopoietic stem cell transplantation as the test intervention harvested from any source of matched sibling donor and serving as a first-line therapy. We included immunosuppressive therapy as comparator with either antithymocyte/antilymphocyte globulin or ciclosporin or a combination of the two. PRIMARY AND SECONDARY OUTCOME MEASURES PLANNED AND FINALLY MEASURED: The primary outcome was overall mortality. Secondary outcomes were treatment-related mortality, graft failure, graft-versus-host disease, no response to immunosuppressive therapy, relapse after initial successful treatment, secondary clonal disease or malignancies, health-related quality of life and performance scores. We identified three prospective non-randomised controlled trials with a study design that was consistent with the principle of 'Mendelian randomisation' in allocating patients to treatment groups. All studies had a high risk of bias due to the study design and were conducted more than 15 years. The pooled HR for overall mortality for the donor group versus the no donor group was 0.95 (95% CI 0.43 to 2.12, p=0.90). There are insufficient and biased data that do not allow any firm conclusions to be made about the comparative effectiveness of first-line allogeneic haematopoietic stem cell transplantation of HLA-matched sibling donors and first-line immunosuppressive therapy of patients with acquired severe aplastic anaemia. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
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Zhu, Hua; He, Jun; Cai, Junchao; Yuan, Xiaoni; Jiang, Hua; Luo, Changying; Wang, Jianmin; Luo, Chengjuan; Pan, Zhijuan; Terasaki, Paul I; Ding, Lixia; Chen, Jing
2014-11-01
Graft failure and survival are the major problems for patients with aplastic anemia undergoing hematopoietic stem cell transplantation (HSCT). Previous studies showed that anti-HLA antibodies negatively impact engraftment in HSCT. This retrospective study of 51 pediatric patients with acquired aplastic anemia who underwent allogeneic HSCT at a single institution between 2006 and 2012 investigated the influence of anti-HLA antibodies on the outcome of HSCT. Serum samples collected before HSCT were tested for the presence of anti-HLA antibodies. Pre-existing anti-HLA antibodies were detected in 54.9% (28/51) of patients, among whom 39.2% (20/51) had anti-HLA class I antibodies. Anti-HLA antibodies were associated with worse five-yr survival (78.6% vs. 100%, p = 0.021) and higher treatment-related mortality (21.4% vs. 0%, p = 0.028) compared with antibody-negative patients. Anti-HLA class I antibody-positive patients had poorer five-yr survival (75.0%) than anti-HLA class I&II antibody-positive and antibody-negative patients (87.5% and 100.0%, respectively, p = 0.039). Presence of anti-HLA class I antibodies (p = 0.024) and older age (10 yr or more; p = 0.027) significantly increased the risk of post-HSCT mortality. Pre-existing anti-HLA antibodies negatively affect the outcome of HSCT in pediatric patients with aplastic anemia. Routine testing for anti-HLA antibodies concurrent with efficient treatment should be conducted prior to HSCT. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
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Pathogenic and host range determinants of the feline aplastic anemia retrovirus.
Riedel, N; Hoover, E A; Dornsife, R E; Mullins, J I
1988-01-01
Feline leukemia virus (FeLV) C-Sarma (or FSC) is a prototype of subgroup C FeLVs, which induce fatal aplastic anemia in outbred specific-pathogen-free (SPF) cats. FeLV C isolates also possess an extended host range in vitro, including an ability, unique among FeLVs, to replicate in guinea pig cells. To identify the viral determinants responsible for the pathogenicity and host range of FSC we constructed a series of proviral DNAs by exchanging gene fragments between FSC and FeLV-61E (or F6A), the latter of which is minimally pathogenic and whose host range in vitro is restricted to feline cells. Transfer of an 886-base-pair (bp) fragment of FSC, encompassing the codons for 73 amino acids at the 3' end of pol (the integrase/endonuclease gene) and the codons for 241 amino acids of the N-terminal portion of env [the extracellular glycoprotein (gp70) gene], into the F6A genome was sufficient to confer onto chimeric viruses the ability to induce fatal aplastic anemia in SPF cats. In contrast, no chimera lacking this sequence induced disease. When assayed in vitro, all chimeric viruses containing the 886-bp fragment of FSC acquired the ability to replicate in heterologous cells, including dog and guinea pig cells. Thus, the pathogenic and the host range determinants of the feline aplastic anemia retrovirus colocalize to a 3' pol-5' env region of the FSC genome and likely reside within a region encoding 241 amino acid residues of the N terminus of the extracellular glycoprotein. Images PMID:2833751
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Government management of two media-facilitated crises involving dioxin contamination of food.
Jacob, Casey J; Lok, Corie; Morley, Katija; Powell, Douglas A
2011-03-01
Incidents become crises through a constant and intense public scrutiny facilitated by the media. Two incidents involving dioxin contamination of food led to crises in Belgium and the Republic of Ireland in 1999 and 2008, respectively. Thought to cause cancer in humans, dioxins reached the food supply in both incidents through the contamination of fat used for animal feed. The food and agricultural industries connected to each incident relied on crisis management activities of federal governments to limit adverse public reaction. Analysis of the management of the two crises by their respective federal governments, and a subsequent review of crisis management literature, led to the development of an effective crisis management model. Such a model, appropriately employed, may insulate industries associated with a crisis against damaged reputations and financial loss.
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Learning through Crisis: The Educator's Role
ERIC Educational Resources Information Center
Patton, Lori D.
2008-01-01
One of the most pressing challenges when facilitating a conversation on human crises is the normalizing push to move forward and avoid living in the past. Unfortunately, when conversations about human crises are ignored or rushed through, the learning process is stunted. Students lose a valuable opportunity to develop empathy and cultural…
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Severe aplastic anaemia and Grave's disease in a paediatric patient.
Kumar, Manjusha; Goldman, Jeffrey
2002-07-01
Severe aplastic anaemia (SAA) is considered to be an autoimmune disorder affecting the haematopoietic cells and most often is idiopathic. An association between SAA and other autoimmune diseases is rare and has been described in adults for eosinophilic fasciitis, thymomas, systemic lupus erythematosus and thyroid disorders. We describe the first paediatric patient with chronic relapsing SAA and Grave's disease. We discuss the difficulty in diagnosis of Grave's disease, the possibility of its manifestation due to withdrawal of immunosuppressants, and issues to consider in the treatment of this disease in the setting of bone marrow failure.
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Honda, Munehiro
2011-01-01
A 52-year-old Japanese woman was examined because of general malaise, weight loss and a lump in her left breast. She was diagnosed with cancer of the left breast and Graves' disease, and was administered methimazole (MMI). A left mastectomy was performed for the breast cancer. She presented with a high fever and peripheral blood examination revealed a severe pancytopenia. She was diagnosed with severe aplastic anemia, and administered G-CSF, however, the treatment was unsuccessful. Thus, oral methyprednisolone and cyclosporin were added. There was a remarkable improvement in the peripheral blood count.
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Global effects of local food-production crises: a virtual water perspective
Tamea, Stefania; Laio, Francesco; Ridolfi, Luca
2016-01-01
By importing food and agricultural goods, countries cope with the heterogeneous global water distribution and often rely on water resources available abroad. The virtual displacement of the water used to produce such goods (known as virtual water) connects together, in a global water system, all countries participating to the international trade network. Local food-production crises, having social, economic or environmental origin, propagate in this network, modifying the virtual water trade and perturbing local and global food availability, quantified in terms of virtual water. We analyze here the possible effects of local crises by developing a new propagation model, parsimonious but grounded on data-based and statistically-verified assumptions, whose effectiveness is proved on the Argentinean crisis in 2008–09. The model serves as the basis to propose indicators of crisis impact and country vulnerability to external food-production crises, which highlight that countries with largest water resources have the highest impact on the international trade, and that not only water-scarce but also wealthy and globalized countries are among the most vulnerable to external crises. The temporal analysis reveals that global average vulnerability has increased over time and that stronger effects of crises are now found in countries with low food (and water) availability. PMID:26804492
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Global effects of local food-production crises: a virtual water perspective.
Tamea, Stefania; Laio, Francesco; Ridolfi, Luca
2016-01-25
By importing food and agricultural goods, countries cope with the heterogeneous global water distribution and often rely on water resources available abroad. The virtual displacement of the water used to produce such goods (known as virtual water) connects together, in a global water system, all countries participating to the international trade network. Local food-production crises, having social, economic or environmental origin, propagate in this network, modifying the virtual water trade and perturbing local and global food availability, quantified in terms of virtual water. We analyze here the possible effects of local crises by developing a new propagation model, parsimonious but grounded on data-based and statistically-verified assumptions, whose effectiveness is proved on the Argentinean crisis in 2008-09. The model serves as the basis to propose indicators of crisis impact and country vulnerability to external food-production crises, which highlight that countries with largest water resources have the highest impact on the international trade, and that not only water-scarce but also wealthy and globalized countries are among the most vulnerable to external crises. The temporal analysis reveals that global average vulnerability has increased over time and that stronger effects of crises are now found in countries with low food (and water) availability.
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Global effects of local food-production crises: a virtual water perspective
NASA Astrophysics Data System (ADS)
Tamea, Stefania; Laio, Francesco; Ridolfi, Luca
2016-01-01
By importing food and agricultural goods, countries cope with the heterogeneous global water distribution and often rely on water resources available abroad. The virtual displacement of the water used to produce such goods (known as virtual water) connects together, in a global water system, all countries participating to the international trade network. Local food-production crises, having social, economic or environmental origin, propagate in this network, modifying the virtual water trade and perturbing local and global food availability, quantified in terms of virtual water. We analyze here the possible effects of local crises by developing a new propagation model, parsimonious but grounded on data-based and statistically-verified assumptions, whose effectiveness is proved on the Argentinean crisis in 2008-09. The model serves as the basis to propose indicators of crisis impact and country vulnerability to external food-production crises, which highlight that countries with largest water resources have the highest impact on the international trade, and that not only water-scarce but also wealthy and globalized countries are among the most vulnerable to external crises. The temporal analysis reveals that global average vulnerability has increased over time and that stronger effects of crises are now found in countries with low food (and water) availability.
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Variations of Young Germans' Informal Conceptions of Financial and Economic Crises Phenomena
ERIC Educational Resources Information Center
Aprea, Carmela; Sappa, Viviana
2014-01-01
The development of a sound understanding of financial and economic crises phenomena must be considered an important goal within the scope of citizenship, economic and social science education. As with every other educational endeavour, this intention requires solid information about what informal conceptions learners hold about this specific…
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Gerull, Sabine; Stern, Martin; Apperley, Jane; Beelen, Dietrich; Brinch, Lorentz; Bunjes, Donald; Butler, Andrew; Ganser, Arnold; Ghavamzadeh, Ardeshir; Koh, Mickey B; Komarnicki, Mieczyslaw; Kröger, Nicolaus; Maertens, Johan; Maschan, Alexei; Peters, Christina; Rovira, Montserrat; Sengeløv, Henrik; Socié, Gerard; Tischer, Johanna; Oneto, Rosi; Passweg, Jakob; Marsh, Judith
2013-01-01
Aplastic anemia is usually treated with immunosuppression or allogeneic transplant, depending on patient and disease characteristics. Syngeneic transplant offers a rare treatment opportunity with minimal transplant-related mortality, and offers an insight into disease mechanisms. We present here a retrospective analysis of all syngeneic transplants for aplastic anemia reported to the European Group for Blood and Marrow Transplantation. Between 1976 and 2009, 88 patients received 113 transplants. Most transplants (n=85) were preceded by a conditioning regimen, 22 of these including anti-thymocyte globulin. About half of transplants with data available (39 of 86) were followed by posttransplant immunosuppression. Graft source was bone marrow in the majority of cases (n=77). Transplant practice changed over time with more transplants with conditioning and anti-thymocyte globulin as well as peripheral blood stem cells performed in later years. Ten year overall survival was 93% with 5 transplant-related deaths. Graft failure occurred in 32% of transplants. Risk of graft failure was significantly increased in transplants without conditioning, and with bone marrow as graft source. Lack of posttransplant immunosuppression also showed a trend towards increased risk of graft failure, while anti-thymocyte globulin did not have an influence. In summary, syngeneic transplant is associated with a significant risk of graft failure when no conditioning is given, but has an excellent long-term outcome. Furthermore, our comparatively large series enables us to recommend the use of pre-transplant conditioning rather than not and possibly to prefer peripheral blood as a stem cell source. PMID:23894010
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Mori, Takehiko; Koh, Hideo; Onishi, Yasushi; Kako, Shinichi; Onizuka, Makoto; Kanamori, Heiwa; Ozawa, Yukiyasu; Kato, Chiaki; Iida, Hiroatsu; Suzuki, Ritsuro; Ichinohe, Tatsuo; Kanda, Yoshinobu; Maeda, Tetsuo; Nakao, Shinji; Yamazaki, Hirohito
2016-04-01
The standard conditioning regimen in allogeneic hematopoietic stem cell transplantation (HSCT) for aplastic anemia from a human leukocyte antigen (HLA)-identical sibling has been high-dose cyclophosphamide (CY 200 mg/kg). In the present study, results for 203 patients with aplastic anemia aged 16 years or older who underwent allogeneic HSCT from HLA-identical siblings were retrospectively analyzed using the registry database of Japan Society for Hematopoietic Cell Transplantation. Conditioning regimens were defined as a (1) high-dose CY (200 mg/kg or greater)-based (n = 117); (2) reduced-dose CY (100 mg/kg or greater, but less than 200 mg/kg)-based (n = 38); and (3) low-dose CY (less than 100 mg/kg)-based (n = 48) regimen. Patient age and the proportion of patients receiving fludarabine were significantly higher in the reduced- and low-dose CY groups than the high-dose CY group. Engraftment was comparable among the groups. Five-year overall survival (OS) tended to be higher in the low-dose CY group [93.0 % (95 % CI 85.1-100.0 %)] than the high-dose CY [84.2 % (95 % CI 77.1-91.3 %)] or reduced-dose CY groups [83.8 % (95 % CI 71.8-95.8 %); P = 0.214]. Age-adjusted OS was higher in the low-dose CY group than the high- and reduced-dose CY groups with borderline significance (P = 0.067). These results suggest that CY dose can safely be reduced without increasing graft rejection by adding fludarabine in allogeneic HSCT for aplastic anemia from an HLA-identical sibling.
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Peinemann, Frank; Grouven, Ulrich; Kröger, Nicolaus; Bartel, Carmen; Pittler, Max H.; Lange, Stefan
2011-01-01
Introduction Acquired severe aplastic anemia (SAA) is a rare and progressive disease characterized by an immune-mediated functional impairment of hematopoietic stem cells. Transplantation of these cells is a first-line treatment option if HLA-matched related donors are available. First-line immunosuppressive therapy may be offered as alternative. The aim was to compare the outcome of these patients in controlled trials. Methods A systematic search was performed in the bibliographic databases MEDLINE, EMBASE, and The Cochrane Library. To show an overview of various outcomes by treatment group we conducted a meta-analysis on overall survival. We evaluated whether studies reported statistically significant factors for improved survival. Results 26 non-randomized controlled trials (7,955 patients enrolled from 1970 to 2001) were identified. We did not identify any RCTs. Risk of bias was high except in 4 studies. Young age and recent year of treatment were identified as factors for improved survival in the HSCT group. Advanced age, SAA without very severe aplastic anemia, and combination of anti-lymphocyte globulin with cyclosporine A were factors for improved survival in the IST group. In 19 studies (4,855 patients), summary statistics were sufficient to be included in meta-analysis. Considerable heterogeneity did not justify a pooled estimate. Adverse events were inconsistently reported and varied significantly across studies. Conclusions Young age and recent year of treatment were identified as factors for improved survival in the transplant group. Advanced age, SAA without very severe aplastic anemia, and combination of anti-lymphocyte globulin with cyclosporine A were factors for improved survival in the immunosuppressive group. Considerable heterogeneity of non-randomized controlled studies did not justify a pooled estimate. Adverse events were inconsistently reported and varied significantly across studies. PMID:21541024
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Gallo, S; Woolfrey, A E; Burroughs, L M; Storer, B E; Flowers, M E D; Hari, P; Pulsipher, M A; Heimfeld, S; Kiem, H-P; Sandmaier, B M; Storb, R
2016-12-01
A total of 21 patients with severe aplastic anemia (SAA) underwent marrow transplantation from HLA-identical siblings following a standard conditioning regimen with cyclophosphamide (50 mg/kg/day × 4 days) and horse antithymocyte globulin (30 mg/kg/day × 3 days). Post-grafting immunosuppression consisted of a short course of methotrexate (MTX) combined with cyclosporine (CSP). The transplant protocol tested the hypothesis that the incidence of chronic GvHD could be reduced by limiting the marrow grafts to ⩽2.5 × 10 8 nucleated marrow cells/kg. None of the patients rejected the graft, all had sustained engraftment and all are surviving at a median of 4 (range 1-8) years after transplantation. Chronic GvHD developed in 16% of patients given ⩽2.5 × 10 8 nucleated marrow cells/kg. Post-grafting immunosuppression has been discontinued in 20 of the 21 patients. In conclusion, limiting the number of transplanted marrow cells may have resulted in minimal improvement in the incidence and severity of chronic GvHD.
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The complex pathophysiology of acquired aplastic anaemia.
Zeng, Y; Katsanis, E
2015-06-01
Immune-mediated destruction of haematopoietic stem/progenitor cells (HSPCs) plays a central role in the pathophysiology of acquired aplastic anaemia (aAA). Dysregulated CD8(+) cytotoxic T cells, CD4(+) T cells including T helper type 1 (Th1), Th2, regulatory T cells and Th17 cells, natural killer (NK) cells and NK T cells, along with the abnormal production of cytokines including interferon (IFN)-γ, tumour necrosis factor (TNF)-α and transforming growth factor (TGF)-β, induce apoptosis of HSPCs, constituting a consistent and defining feature of severe aAA. Alterations in the polymorphisms of TGF-β, IFN-γ and TNF-α genes, as well as certain human leucocyte antigen (HLA) alleles, may account for the propensity to immune-mediated killing of HSPCs and/or ineffective haematopoiesis. Although the inciting autoantigens remain elusive, autoantibodies are often detected in the serum. In addition, recent studies provide genetic and molecular evidence that intrinsic and/or secondary deficits in HSPCs and bone marrow mesenchymal stem cells may underlie the development of bone marrow failure. © 2015 British Society for Immunology.
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Assessing Chaos in Sickle Cell Anemia Crises
NASA Astrophysics Data System (ADS)
Harris, Wesley; Le Floch, Francois
2006-11-01
Recent developments in sickle cell research and blood flow modeling allow for new interpretations of the sickle cell crises. With an appropriate set of theoretical and empirical equations describing the dynamics of the red cells in their environment, and the response of the capillaries to major changes in the rheology, a complete mathematical system has been derived. This system of equations is believed to be of major importance to provide new and significant insight into the causes of the disease and related crises. With simulations, it has been proven that the system transition from a periodic solution to a chaotic one, which illustrates the onset of crises from a regular blood flow synchronized with the heart beat. Moreover, the analysis of the effects of various physiological parameters exposes the potential to control chaotic solutions, which, in turn, could lead to the creation of new and more effective treatments for sickle cell anemia. .
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ERIC Educational Resources Information Center
Emily Hall Tremaine Foundation, Inc., Hartford, CT.
This learning guide on improving responses to individual and family crises is part of a series of learning guides developed for competency-based adult consumer and homemaking education programs in community colleges, adult education centers, community centers, and the workplace. Focus is on the connections among personal, family, and job…
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School District Policies for Response to Death-Related Crises: Fact or Fiction?
ERIC Educational Resources Information Center
Christenberry, Nola J.; Burns, John L.
Findings of a literature review of school policies and procedures for school-based responses to death-related crises are presented in this paper. A rationale and guidelines for policy development and examples of practices for dealing with death-related incidents--such as suicide, homicide, drug overdose, and accidents--are described. Following an…
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Acquired Aplastic Anemia: What Have We Learned and What Is in the Horizon?
Savaşan, Süreyya
2018-06-01
Acquired aplastic anemia (aAA) characterized by peripheral pancytopenia and bone marrow aplasia is a rare and serious disorder. Differential diagnosis includes constitutional bone marrow failure syndromes and myelodysplastic disorders. Autoimmune reaction to altered hematopoietic stem cells highlights the underlying mechanism. Matched related donor allogeneic hematopoietic stem cell transplantation is the ideal pediatric treatment; alternative approaches include immunosuppressive therapy and use of eltrombopag. Progression to clonal disorders can occur. Recently, alternative donor hematopoietic stem cell transplantation outcomes have significantly improved. Despite advances, aAA continues to be a challenge for hematologists. Copyright © 2018 Elsevier Inc. All rights reserved.
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Barone, Angelica; Lucarelli, Annunziata; Onofrillo, Daniela; Verzegnassi, Federico; Bonanomi, Sonia; Cesaro, Simone; Fioredda, Francesca; Iori, Anna Paola; Ladogana, Saverio; Locasciulli, Anna; Longoni, Daniela; Lanciotti, Marina; Macaluso, Alessandra; Mandaglio, Rosalba; Marra, Nicoletta; Martire, Baldo; Maruzzi, Matteo; Menna, Giuseppe; Notarangelo, Lucia Dora; Palazzi, Giovanni; Pillon, Marta; Ramenghi, Ugo; Russo, Giovanna; Svahn, Johanna; Timeus, Fabio; Tucci, Fabio; Cugno, Chiara; Zecca, Marco; Farruggia, Piero; Dufour, Carlo; Saracco, Paola
2015-06-01
Acquired aplastic anemia (AA) is a rare heterogeneous disease characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3/million inhabitants/year, in Europe, but higher in East Asia. Survival in severe aplastic anemia (SAA) has markedly improved in the past 2 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. In SAA hematopoietic stem cell transplant (HSCT) from a matched sibling donor (MSD) is the treatment of choice. If a MSD is not available, the options include immunosuppressive therapy (IST) or unrelated donor HSCT. The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary, evidence-based document issued by a group of pediatric hematologists was discussed, modified and approved during a series of "Consensus Conferences" according to procedures previously validated by the AIEOP Board. The guidelines highlight the importance of referring pediatric patients with AA to pediatric centers with long experience in diagnosis, differential diagnosis, management, supportive care and follow-up of AA. Copyright © 2015. Published by Elsevier Inc.
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Mesenchymal Stem Cell Benefits Observed in Bone Marrow Failure and Acquired Aplastic Anemia
Gonzaga, Vivian Fonseca; Lisboa, Gustavo Sabino; Frare, Eduardo Osório
2017-01-01
Acquired aplastic anemia (AA) is a type of bone marrow failure (BMF) syndrome characterized by partial or total bone marrow (BM) destruction resulting in peripheral blood (PB) pancytopenia, which is the reduction in the number of red blood cells (RBC) and white blood cells (WBC), as well as platelets (PLT). The first-line treatment option of AA is given by hematopoietic stem cell (HSCs) transplant and/or immunosuppressive (IS) drug administration. Some patients did not respond to the treatment and remain pancytopenic following IS drugs. The studies are in progress to test the efficacy of adoptive cellular therapies as mesenchymal stem cells (MSCs), which confer low immunogenicity and are reliable allogeneic transplants in refractory severe aplastic anemia (SAA) cases. Moreover, bone marrow stromal cells (BMSC) constitute an essential component of the hematopoietic niche, responsible for stimulating and enhancing the proliferation of HSCs by secreting regulatory molecules and cytokines, providing stimulus to natural BM microenvironment for hematopoiesis. This review summarizes scientific evidences of the hematopoiesis improvements after MSC transplant, observed in acquired AA/BMF animal models as well as in patients with acquired AA. Additionally, we discuss the direct and indirect contribution of MSCs to the pathogenesis of acquired AA. PMID:29333168
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Heckl, Dirk; Wicke, Daniel C; Brugman, Martijn H; Meyer, Johann; Schambach, Axel; Büsche, Guntram; Ballmaier, Matthias; Baum, Christopher; Modlich, Ute
2011-04-07
Thpo/Mpl signaling plays an important role in the maintenance of hematopoietic stem cells (HSCs) in addition to its role in megakaryopoiesis. Patients with inactivating mutations in Mpl develop thrombocytopenia and aplastic anemia because of progressive loss of HSCs. Yet, it is unknown whether this loss of HSCs is an irreversible process. In this study, we used the Mpl knockout (Mpl(-/-)) mouse model and expressed Mpl from newly developed lentiviral vectors specifically in the physiologic Mpl target populations, namely, HSCs and megakaryocytes. After validating lineage-specific expression in vivo using lentiviral eGFP reporter vectors, we performed bone marrow transplantation of transduced Mpl(-/-) bone marrow cells into Mpl(-/-) mice. We show that restoration of Mpl expression from transcriptionally targeted vectors prevents lethal adverse reactions of ectopic Mpl expression, replenishes the HSC pool, restores stem cell properties, and corrects platelet production. In some mice, megakaryocyte counts were atypically high, accompanied by bone neo-formation and marrow fibrosis. Gene-corrected Mpl(-/-) cells had increased long-term repopulating potential, with a marked increase in lineage(-)Sca1(+)cKit(+) cells and early progenitor populations in reconstituted mice. Transcriptome analysis of lineage(-)Sca1(+)cKit(+) cells in Mpl-corrected mice showed functional adjustment of genes involved in HSC self-renewal.
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Betensky, Marisol; Babushok, Daria; Roth, Jacquelyn J; Mason, Philip J; Biegel, Jaclyn A; Busse, Tracy M; Li, Yimei; Lind, Curt; Papazoglou, Anna; Monos, Dimitri; Podsakoff, Gregory; Bessler, Monica; Olson, Timothy S
2016-01-01
Acquired aplastic anemia (aAA) results from the T cell-mediated autoimmune destruction of hematopoietic stem cells. Factors predicting response to immune suppression therapy (IST) or development of myelodysplastic syndrome (MDS) are beginning to be elucidated. Our recent data suggest most patients with aAA treated with IST develop clonal somatic genetic alterations in hematopoietic cells. One frequent acquired abnormality is copy-number neutral loss of heterozygosity on chromosome 6p (6p CN-LOH) involving the human leukocyte antigen (HLA) locus. We hypothesized that because 6p CN-LOH clones may arise from selective pressure to escape immune surveillance through deletion of HLA alleles, the development of 6p CN-LOH may affect response to IST. We used single nucleotide polymorphism array genotyping and targeted next-generation sequencing of HLA alleles to assess frequency of 6p CN-LOH, identity of HLA alleles lost through 6p CN-LOH, and impact of 6p CN-LOH on response to IST. 6p CN-LOH clones were present in 11.3% of patients, remained stable over time, and were not associated with development of MDS-defining cytogenetic abnormalities. Notably, no patient with 6p CN-LOH treated with IST achieved a complete response. In summary, clonal 6p CN-LOH in aAA defines a unique subgroup of patients that may provide insights into hematopoietic clonal evolution. Copyright © 2016 Elsevier Inc. All rights reserved.
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Betensky, Marisol; Babushok, Daria; Roth, Jacquelyn J.; Mason, Philip J; Biegel, Jaclyn A.; Busse, Tracy M; Li, Yimei; Lind, Curt; Papazoglou, Anna; Monos, Dimitri; Podsakoff, Gregory; Bessler, Monica; Olson, Timothy S.
2015-01-01
Acquired aplastic anemia (aAA) results from the T cell-mediated autoimmune destruction of hematopoietic stem cells. Factors predicting response to immune suppression therapy (IST) or development of myelodysplastic syndrome (MDS) are beginning to be elucidated. Our recent data suggest most patients with aAA treated with IST develop clonal somatic genetic alterations in hematopoietic cells. One frequent acquired abnormality is copy-number neutral loss of heterozygosity on chromosome 6p (6p CN-LOH) involving the human leukocyte antigen (HLA) locus. We hypothesized that because 6p CN-LOH clones may arise from selective pressure to escape immune surveillance through deletion of HLA alleles, the development of 6p CN-LOH may affect response to IST. We used single nucleotide polymorphism array genotyping and targeted next-generation sequencing of HLA alleles to assess frequency of 6p CN-LOH, identity of HLA alleles lost through 6p CN-LOH, and impact of 6p CN-LOH on response to IST. 6p CN-LOH clones were present in 11.3% of patients, remained stable over time, and were not associated with development of MDS-defining cytogenetic abnormalities. Notably, no patient with 6p CN-LOH treated with IST achieved a complete response. In summary, clonal 6p CN-LOH in aAA defines a unique subgroup of patients that may provide insights into hematopoietic clonal evolution. PMID:26702937
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ERIC Educational Resources Information Center
Emily Hall Tremaine Foundation, Inc., Hartford, CT.
This competency-based secondary learning guide on improving responses to crises is part of a series that are adaptations of guides developed for adult consumer and homemaking education programs. The guides provide students with experiences that help them learn to do the following: make decisions; use creative approaches to solve problems;…
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EPA guidance on mental health and economic crises in Europe.
Martin-Carrasco, M; Evans-Lacko, S; Dom, G; Christodoulou, N G; Samochowiec, J; González-Fraile, E; Bienkowski, P; Gómez-Beneyto, M; Dos Santos, M J H; Wasserman, D
2016-03-01
This European Psychiatric Association (EPA) guidance paper is a result of the Working Group on Mental Health Consequences of Economic Crises of the EPA Council of National Psychiatric Associations. Its purpose is to identify the impact on mental health in Europe of the economic downturn and the measures that may be taken to respond to it. We performed a review of the existing literature that yields 350 articles on which our conclusions and recommendations are based. Evidence-based tables and recommendations were developed through an expert consensus process. Literature dealing with the consequences of economic turmoil on the health and health behaviours of the population is heterogeneous, and the results are not completely unequivocal. However, there is a broad consensus about the deleterious consequences of economic crises on mental health, particularly on psychological well-being, depression, anxiety disorders, insomnia, alcohol abuse, and suicidal behaviour. Unemployment, indebtedness, precarious working conditions, inequalities, lack of social connectedness, and housing instability emerge as main risk factors. Men at working age could be particularly at risk, together with previous low SES or stigmatized populations. Generalized austerity measures and poor developed welfare systems trend to increase the harmful effects of economic crises on mental health. Although many articles suggest limitations of existing research and provide suggestions for future research, there is relatively little discussion of policy approaches to address the negative impact of economic crises on mental health. The few studies that addressed policy questions suggested that the development of social protection programs such as active labour programs, social support systems, protection for housing instability, and better access to mental health care, particularly at primary care level, is strongly needed.
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Baris, Hagit N; Weisz Hubshman, Monika; Bar-Sever, Zvi; Kornreich, Liora; Shkalim Zemer, Vered; Cohen, Ian J
2016-09-01
Bone crises in type 1 Gaucher disease are reported in long bones and occasionally in weight bearing bones and other bones, but rarely in small bones of the hands and feet. We retrospectively examined the incidence of bone pain in patients followed at the Rabin Medical Center, Israel, before and following the initiation of enzyme replacement therapy (ERT) and evaluated them for bone crises. Of 100 type I Gaucher disease patients, 30 (30%) experienced one or more bone crises. Small bone crises represented 31.5% of all bone crises and were always preceded by crises in other bones. While the incidence of long bone crises reduced after the initiation of ERT, small bone crises increased. Almost 60% of patients with bone crises were of the N370S/84GG genotype suggesting a greater susceptibility of N370S/84GG patients to severe bone complications. These patients also underwent the greatest number of splenectomies (70.6% of splenectomised patients). Splenectomised patients showed a trend towards increased long and small bone crises after surgery. Active investigation of acute pain in the hands and feet in patients in our cohort has revealed a high incidence of small bone crises. Physicians should consider imaging studies to investigate unexplained pain in these areas. Copyright © 2015 Elsevier Inc. All rights reserved.
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Umbilical Cord Blood Transplantation From Unrelated Donors
2018-02-17
Acute Leukemia; Immune Deficiency Disorder; Congenital Hematological Disorder; Metabolism Disorder; Aplastic Anemia; Myelodysplastic Syndromes; Chronic Leukemia; Lymphoma; Multiple Myeloma; Solid Tumor
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The Unfinished Revolution: Einstein's revenge
NASA Astrophysics Data System (ADS)
Beichler, James
2007-04-01
Thomas Kuhn defined the characteristics of scientific revolutions based upon his knowledge of the first and second Scientific Revolutions. He concluded that such revolutions are the result of crises in science. However, he missed some important clues of how revolutions develop. Instead of looking at crises, we should look at the major trends in scientific and human thought prior to the revolutions and then we could gain a better understanding of how scientific revolutions emerge from the normal course of scientific evolution. Instead of defining revolutions by the crises that precede them, revolutions actually emerge from the successes of previous science while each revolution contains the seeds for the next revolution that follows. These seeds eventually grow into the crises that trigger revolutions. Under these circumstances, it can be shown that the space-time revolution of relativity theory was never completed, thus laying the foundations for the next revolution in science. Knowing this, we can determine if we have we already entered the pre-revolutionary period of the Third Scientific Revolution.
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Families in the context of macroeconomic crises: A systematic review.
Fonseca, Gabriela; Cunha, Diana; Crespo, Carla; Relvas, Ana Paula
2016-09-01
The present study is a systematic review of empirical literature from the last 35 years on families' responses to economic distress in the context of macroeconomic crises. Thirty-nine studies published between 1983 and 2015 in 12 countries were identified, resulting in 3 main findings. First, economic distress was associated with negative changes in family dynamics, specifically couple relationships and parenting. Second, protective factors were found to buffer the adverse effects of economic distress on family and individual outcomes. Third, the results suggest that individual responses to macroeconomic crises may be moderated by sex. Implications for future research encompass using validated assessment instruments, including participants beyond 2-parent families with adolescent children and conducting both longitudinal and qualitative studies that focus on the processes and meanings of adaptation within this risk context. Conclusions highlighted the need to assist families dealing with macroeconomic crises' demands, encouraging the development and validation of macrosystemic intervention programs. (PsycINFO Database Record (c) 2016 APA, all rights reserved).
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Varma, Neelam; Varma, Subhash; Marwaha, Ram Kumar; Malhotra, Pankaj; Bansal, Deepak; Malik, Kiran; Kaur, Sukhdeep; Garewal, Gurjeevan
2006-07-01
A large number of patients diagnosed with bone marrow failure syndromes (BMFS), comprising aplastic anaemia (AA) and myelodysplastic syndromes (MDS), remain aetiologically uncharacterized worldover, especially in resource constrained set up. We carried out this study to identify a few constitutional causes in BMFS patients attending a tertiary care hospital in north India. Peripheral blood lymphocyte cultures were performed (with and without clastogens) in a cohort of 135 consecutive BMFS patients, in order to detect Fanconi anaemia (FA), Down's syndrome (+21), trisomy 8 (+8) and monosomy 7 (-7). Constitutional factors were detected in 17 (12.6%) patients. FA defect was observed in 24.07 percent (13/54), 16.66 percent (1/6) and 2.85 percent (1/35) paediatric aplastic anaemia, paediatric MDS and adult MDS patients respectively. Down's syndrome was detected in 5.00 percent (2/40) adult aplastic anaemia patients. None of the patients revealed trisomy 8 or monosomy 7. Presence of an underlying factor determines appropriate management, prognostication, family screening and genetic counselling of BMFS patients. Special tests required to confirm or exclude constitutional aetiological factors are not available to majority of the patients in our country. Diepoxybutane (DEB) test yielded better results than mitomycin C (MMC) test in our experience.
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Cui, Qingya; Sha, Pingping; Chen, Haifei; Shen, Hongshi; Qin, Longmei; Li, Zhengyang; Wu, Tianqin; Wang, Zhaoyue
2018-01-01
Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine (CsA) is the standard treatment for children with severe aplastic anemia (SAA) with no human leukocyte antigen-matched siblings. Due to the unavailability of horse ATG in China, porcine antilymphocyte globulin (p-ALG), which is less expensive and more effective than rabbit ATG, is widely used. We sought to evaluate the efficacy and safety profile of modified IST with p-ALG plus delayed CsA at day 21 in 50 SAA children. Eighteen SAA patients who progressed from nonsevere aplastic anemia (NSAA) were classified as SAA-II; the other 32 patients were classified as SAA-I. Overall response (OR) rates at 3, 6 and 12 months were 56, 64 and 62%, respectively. The 10-year overall survival (OS) rate and disease-free survival (DFS) rate were 80 and 56%. The OR, OS and DFS rates in the SAA-I group were clearly better than those in the SAA-II group. Death rate from infection within 30 days was 4%. Modified IST with p-ALG plus delayed CsA is a reliable and well-tolerated treatment for children with SAA, and reduces early death due to infection. Modified IST is more suitable for children with SAA-I.
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Yin and Yang of mesenchymal stem cells and aplastic anemia
Broglie, Larisa; Margolis, David; Medin, Jeffrey A
2017-01-01
Acquired aplastic anemia (AA) is a bone marrow failure syndrome characterized by peripheral cytopenias and bone marrow hypoplasia. It is ultimately fatal without treatment, most commonly from infection or hemorrhage. Current treatments focus on suppressing immune-mediated destruction of bone marrow stem cells or replacing hematopoietic stem cells (HSCs) by transplantation. Our incomplete understanding of the pathogenesis of AA has limited development of targeted treatment options. Mesenchymal stem cells (MSCs) play a vital role in HSC proliferation; they also modulate immune responses and maintain an environment supportive of hematopoiesis. Some of the observed clinical manifestations of AA can be explained by mesenchymal dysfunction. MSC infusions have been shown to be safe and may offer new approaches for the treatment of this disorder. Indeed, infusions of MSCs may help suppress auto-reactive, T-cell mediated HSC destruction and help restore an environment that supports hematopoiesis. Small pilot studies using MSCs as monotherapy or as adjuncts to HSC transplantation have been attempted as treatments for AA. Here we review the current understanding of the pathogenesis of AA and the function of MSCs, and suggest that MSCs should be a target for further research and clinical trials in this disorder. PMID:29321823
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Iliac crest tap; Sternal tap; Leukemia - bone marrow aspiration; Aplastic anemia - bone marrow aspiration; Myelodysplastic syndrome - bone marrow aspiration; Thrombocytopenia - bone marrow aspiration; Myelofibrosis - bone marrow aspiration
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Sakaguchi, Hirotoshi; Nishio, Nobuhiro; Hama, Asahito; Kawashima, Nozomu; Wang, Xinan; Narita, Atsushi; Doisaki, Sayoko; Xu, Yinyan; Muramatsu, Hideki; Yoshida, Nao; Takahashi, Yoshiyuki; Kudo, Kazuko; Moritake, Hiroshi; Nakamura, Kazuhiro; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji
2014-01-01
Predicting the response to immunosuppressive therapy could provide useful information to help the clinician define treatment strategies for patients with aplastic anemia. In our current study, we evaluated the relationship between telomere length of lymphocytes at diagnosis and the response to immunosuppressive therapy in 64 children with aplastic anemia, using flow fluorescence in situ hybridization. Median age of patients was ten years (range 1.5–16.2 years). Severity of the disease was classified as very severe in 23, severe in 21, and moderate in 20 patients. All patients were enrolled in multicenter studies using antithymocyte globulin and cyclosporine. The response rate to immunosuppressive therapy at six months was 52% (33 of 64). The probability of 5-year failure-free survival and overall survival were 56% (95% confidence interval (CI): 41–69%) and 97% (95%CI: 87–99%), respectively. Median telomere length in responders was −0.4 standard deviation (SD) (−2.7 to +3.0 SD) and −1.5 SD (−4.0 to +1.6 (SD)) in non-responders (P<0.001). Multivariate analysis showed that telomere length shorter than −1.0 SD (hazard ratio (HR): 22.0; 95%CI: 4.19–115; P<0.001), platelet count at diagnosis less than 25×109/L (HR: 13.9; 95%CI: 2.00–96.1; P=0.008), and interval from diagnosis to immunosuppressive therapy longer than 25 days (HR: 4.81; 95%CI: 1.15–20.1; P=0.031) were the significant variables for poor response to immunosuppressive therapy. Conversely to what has been found in adult patients, measurement of the telomere length of lymphocytes at diagnosis is a promising assay in predicting the response to immunosuppressive therapy in children with aplastic anemia. PMID:24816243
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Yang, W R; Jing, L P; Zhou, K; Peng, G X; Li, Y; Ye, L; Li, Y; Li, J P; Fan, H H; Song, L; Zhao, X; Yang, Y; Zhang, F K; Zhang, L
2016-05-14
To analyze the clinical characteristics and to evaluate immunosuppressive therapy (IST) response and survival in hepatitis-associated aplastic anemia (HAAA). We retrospectively analyzed clinical characteristics, IST response, long-term survival and clonal evolution in 41 HAAA patients, and compared those with age and bone marrow failure matched idiopathic aplastic anemia (IAA) patients. The prevalence of HAAA among cases of SAA was 4.34% (41/944). The proportion of VSAA in HAAA cases was significantly higher than IAA (65.9% vs 39.4%, P=0.001). There was no significant difference in the prevalence of hemorrhage and infections between HAAA and IAA patients, but the duration of infection persistence in HAAA group was much longer than IAA group [21 (4-100) d vs 13 (3-139) d, P=0.048]. The absolute counts of CD3(+) T-cell, CD3(+)CD4(+)T-cell, CD3(+)CD8(+)T-cell and ratio of CD4(+) T-cell/CD8(+) T-cell in HAAA were significant lower than that in IAA patients. However, the percentage of CD3(+)CD8(+)T-cell in HAAA was significant higher than that in IAA (P <0.05). The total response in HAAA and IAA patients treated with IST were 34.1% vs 34.1% (P=1.000), 56.1% vs 53.7% (P=0.787), and 73.2% vs 68.3% (P=0.558) at 3, 6, 12 months after IST, respectively. There were no significant difference in 5-year overall survival and event-free survival between HAAA and IAA patients (90% vs 87.1%, P=0.700; 71.9% vs 62.4%, P=0.450). HAAA was a rare distinct variant of aplastic anemia with more severe bone marrow failure and more severe imbalance of the T cell immune system than IAA. Treatment outcomes were comparable in patients with HAAA and IAA.
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DeZern, Amy E.; Petri, Michelle; Drachman, Daniel B.; Kerr, Doug; Hammond, Edward R.; Kowalski, Jeanne; Tsai, Hua-Ling; Loeb, David M.; Anhalt, Grant; Wigley, Fredrick; Jones, Richard J.; Brodsky, Robert A.
2011-01-01
High-dose cyclophosphamide has long been used an anticancer agent, a conditioning regimen for hematopoietic stem cell transplantation and as potent immunosuppressive agent in autoimmune diseases including aplastic anemia. High-dose cyclophosphamide is highly toxic to lymphocytes but spares hematopoietic stem cells because of their abundant levels of aldehyde dehydrogenase, the major mechanism of cyclophosphamide inactivation. High dose cyclophosphamide therapy induces durable remissions in most patients with acquired aplastic anemia. Moreover, high-dose cyclophosphamide without hematopoietic stem cell rescue has shown activity in a variety of other severe autoimmune diseases. Here we review the history of cyclophosphamide as is applies to aplastic anemia (AA) and other autoimmune diseases. Included here are the historical data from early patients treated for AA as well as an observational retrospective study in a single tertiary care hospital. This latter component was designed to assess the safety and efficacy of high-dose cyclophosphamide therapy without stem cell rescue in patients with refractory autoimmune diseases. We analyzed fully the 140 patients with severe, progressive autoimmune diseases treated. All patients discussed here received cyclophosphamide, 50 mg/kg per day for 4 consecutive days. Response, relapse and overall survival were measured. Response was defined as a decrease in disease activity in conjunction with a decrease or elimination of immune modulating drugs. Relapse was defined as worsening disease activity and/or a requirement of an increase in dose of, or administration of new, immunosuppressive medications. Hematologic recovery occurred in all patients. The overall response rate of the was 95%, and 44% of those patients remain progression-free with a median follow up time of 36 (range 1–120) months for the 140 patients analyzed together. The overall actuarial and event free survival across all diseases at 60 months is 90.7% and 20.6%, respectively. High- dose cyclophosphamide without stem cell rescue is well-tolerated and induces a high rate of remissions in severe autoimmune diseases. PMID:21358440
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Gadalla, Shahinaz M; Wang, Tao; Haagenson, Michael; Spellman, Stephen R; Lee, Stephanie J; Williams, Kirsten M; Wong, Jason Y; De Vivo, Immaculata; Savage, Sharon A
2015-02-10
Telomeres protect chromosome ends and are markers of cellular aging and replicative capacity. To evaluate the association between recipient and donor pretransplant leukocyte telomere length with outcomes after unrelated donor allogeneic hematopoietic cell transplantation (HCT) for patients with severe aplastic anemia. The study included 330 patients (235 acquired, 85 Fanconi anemia, and 10 Diamond-Blackfan anemia) and their unrelated donors who had pre-HCT blood samples and clinical and outcome data available at the Center for International Blood and Marrow Transplant Research. Patients underwent HCT between 1989 and 2007 in 84 centers and were followed-up to March 2013. Recipient and donor pre-HCT leukocyte telomere length classified into long (third tertile) and short (first and second tertiles combined) based on donor telomere length distribution. Overall survival, neutrophil recovery, and acute and chronic graft-vs-host disease, as ascertained by transplant centers through regular patient follow-up. Longer donor leukocyte telomere length was associated with higher survival probability (5-year overall survival, 56%; number at risk, 57; cumulative deaths, 50) than shorter donor leukocyte telomere length (5-year overall survival, 40%; number at risk, 71; cumulative deaths, 128; P = .009). The association remained statistically significant after adjusting for donor age, disease subtype, Karnofsky performance score, graft type, HLA matching, prior aplastic anemia therapy, race/ethnicity, and calendar year of transplant (hazard ratio [HR], 0.61; 95% CI, 0.44-0.86). Similar results were noted in analyses stratified on severe aplastic anemia subtype, recipient age, HLA matching, calendar year of transplant, and conditioning regimen. There was no association between donor telomere length and neutrophil engraftment at 28 days (cumulative incidence, 86% vs 85%; HR, 0.94; 95% CI, 0.73-1.22), acute graft-vs-host disease grades III-IV at 100 days (cumulative incidence, 22% vs 28%; HR, 0.77; 95% CI, 0.48-1.23), or chronic graft-vs-host disease at 1-year (cumulative incidence, 28% vs 30%; HR, 0.81; 95% CI, 0.53-1.24) for long vs short, respectively. Pretransplant leukocyte telomere length in the recipients was not associated with posttransplant survival (HR, 0.91; 95% CI, 0.64-1.30). Longer donor leukocyte telomere length was associated with increased 5-year survival in patients who received HCT for severe aplastic anemia. Patient leukocyte telomere length was not associated with survival. The results of this observational study suggest that donor leukocyte telomere length may have a role in long-term posttransplant survival.
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A Role for Science in Responding to Health Crises
Brothers, Reginald; Murata, Christina E.
2016-01-01
The Department of Homeland Security's (DHS) Science and Technology (S&T) Directorate plays a role in public health that extends beyond biodefense. These responsibilities were exercised as part of the 2014-16 Ebola outbreak, leading to productive and beneficial contributions to the international public health response and improved operations in the United States. However, we and others have identified numerous areas for improvement. Based on our successes and lessons learned, we propose a number of ways that DHS, the interagency, and academia can act now to ensure improved responses to future public health crises. These include pre-developing scientific capabilities to respond agnostically to threats, and disease-specific master question lists to organize and inform initial efforts. We are generating DHS-specific playbooks and tools for anticipating future needs and capturing requests from DHS components and our national and international partners, where efforts will also be used to refine and exercise communication and information-sharing practices. These experiences and improvement efforts have encouraged discussions on the role of science in developing government policy, specifically responding to public health crises. We propose specific considerations for both scientists and government decision makers to ensure that the best available science is incorporated into policy and operational decisions to facilitate highly effective responses to future health crises. PMID:27482881
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Resilience of natural gas networks during conflicts, crises and disruptions.
Carvalho, Rui; Buzna, Lubos; Bono, Flavio; Masera, Marcelo; Arrowsmith, David K; Helbing, Dirk
2014-01-01
Human conflict, geopolitical crises, terrorist attacks, and natural disasters can turn large parts of energy distribution networks offline. Europe's current gas supply network is largely dependent on deliveries from Russia and North Africa, creating vulnerabilities to social and political instabilities. During crises, less delivery may mean greater congestion, as the pipeline network is used in ways it has not been designed for. Given the importance of the security of natural gas supply, we develop a model to handle network congestion on various geographical scales. We offer a resilient response strategy to energy shortages and quantify its effectiveness for a variety of relevant scenarios. In essence, Europe's gas supply can be made robust even to major supply disruptions, if a fair distribution strategy is applied.
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The synergy of the refugee crisis and the financial crisis in Greece: Impact on mental health.
Anagnostopoulos, Dimitris C; Giannakopoulos, George; Christodoulou, Nikos G
2017-06-01
The current global financial crisis that started in 2008 resulted in a significant decline in global trade, slowing/reversing economic growth worldwide, and a dramatic increase in public sector debt. At the same time, the global migrant/refugee crisis has reached extreme rates, with millions of people being forced to abandon their homes and communities because of war, political violence or related threats. There is a broad consensus about the deleterious consequences of these crises on psychological well-being, depression, anxiety disorders, insomnia, alcohol abuse and suicidal behavior. Although the separate consequences of economic recession and immigration are extensively discussed in previous research, we know very little about the processes through which the intersection of economic crisis and migrant crisis contributes to the vulnerabilities of natives and migrants during these crises. Of particular concern is the status of children, adolescents and their families, who constitute one of the most vulnerable groups in society. To discuss the contexts that economic and migrant crises shape and suggest possible effects of this intersection on mental health risks, especially among children, adolescents and their families, through reflecting on the recent experience in Greece. Review of the literature and critical analysis of the effects of the confluent crises. The interactive effects of these two crises need further exploration. Novel and diverse models of psychological understanding need to be developed in order to manage the effects of the confluent crises. The role of mental health professionals is crucial in this respect, offering culturally flexible, accommodating and empathetic approaches, allowing healing and acceptance in the face of adversity.
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Thies, Bastian; Meyer-Schwesinger, Catherine; Lamp, Jessica; Schweizer, Michaela; Koeller, David M; Ullrich, Kurt; Braulke, Thomas; Mühlhausen, Chris
2013-10-01
The metabolic disorder glutaric aciduria type 1 (GA1) is caused by deficiency of the mitochondrial glutaryl-CoA dehydrogenase (GCDH), leading to accumulation of the pathologic metabolites glutaric acid (GA) and 3-hydroxyglutaric acid (3OHGA) in blood, urine and tissues. Affected patients are prone to metabolic crises developing during catabolic conditions, with an irreversible destruction of striatal neurons and a subsequent dystonic-dyskinetic movement disorder. The pathogenetic mechanisms mediated by GA and 3OHGA have not been fully characterized. Recently, we have shown that GA and 3OHGA are translocated through membranes via sodium-dependent dicarboxylate cotransporter (NaC) 3, and organic anion transporters (OATs) 1 and 4. Here, we show that induced metabolic crises in Gcdh(-/-) mice lead to an altered renal expression pattern of NaC3 and OATs, and the subsequent intracellular GA and 3OHGA accumulation. Furthermore, OAT1 transporters are mislocalized to the apical membrane during metabolic crises accompanied by a pronounced thinning of proximal tubule brush border membranes. Moreover, mitochondrial swelling and increased excretion of low molecular weight proteins indicate functional tubulopathy. As the data clearly demonstrate renal proximal tubule alterations in this GA1 mouse model during induced metabolic crises, we propose careful evaluation of renal function in GA1 patients, particularly during acute crises. Further studies are needed to investigate if these findings can be confirmed in humans, especially in the long-term outcome of affected patients. Copyright © 2013 Elsevier B.V. All rights reserved.
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How I treat acquired aplastic anemia
Young, Neal S.
2012-01-01
Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive biologics and drugs, and supportive care. However, management of SAA patients remains challenging, both acutely in addressing the immediate consequences of pancytopenia and in the long term because of the disease's natural history and the consequences of therapy. Recent insights into pathophysiology have practical implications. We review key aspects of differential diagnosis, considerations in the choice of first- and second-line therapies, and the management of patients after immunosuppression, based on both a critical review of the recent literature and our large personal and research protocol experience of bone marrow failure in the Hematology Branch of the National Heart, Lung, and Blood Institute. PMID:22517900
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Shamshad, Ghassan Umair; Ahmed, Suhaib; Bhatti, Farhat Abbas; Ali, Nadir
2012-12-01
To determine the frequency of mixed donor chimerism in patients of non-malignant haematological diseases after allogeneic bone marrow transplant. A cross-sectional, observational study. Department of Haematology, Armed Forces Institute of Pathology (AFIP), Rawalpindi, from July 2010 to June 2011. Donor chimerism was assessed in patients of aplastic anaemia and beta-thalassaemia major who underwent allogeneic bone marrow transplantation (BMT). Peripheral blood samples were used to assess chimerism status by analysis of short tandem repeats (STR). In patients where pre-transplant blood sample was not available, swab of buccal mucosa was used for pre-transplant STR profile. A standard set of primers for STR markers were used and the amplified DNA was resolved by gel electrophoresis and stained with silver nitrate. The percentage of donor origin DNA was estimated by densitometer. Out of 84 patients, 52 (62%) were males, while 32 (38%) were females. In patients of beta-thalassaemia major, 31 (62%) developed mixed donor chimerism (MC), 13 (26%) developed complete donor chimerism (CC) and 6 (12%) had graft failure. In aplastic anaemia, 17 patients (50%) achieved MC, 13 (38.2%) had CC and 4 (11.8%) developed graft failure. The combined frequency of mixed donor chimerism for both the diseases was 58.3%. D3S1358 was the most informative STR marker in these patients. Majority of the studied patients developed mixed donor chimerism following bone marrow transplantation, whereas only a minor percentage of the patients had graft failure. Analysis of D3S1358 was the most informative in assessing donor chimerism in patients who underwent BMT.
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Human resource crises in German hospitals--an explorative study.
Schermuly, Carsten C; Draheim, Michael; Glasberg, Ronald; Stantchev, Vladimir; Tamm, Gerrit; Hartmann, Michael; Hessel, Franz
2015-05-28
The complexity of providing medical care in a high-tech environment with a highly specialized, limited labour force makes hospitals more crisis-prone than other industries. An effective defence against crises is only possible if the organizational resilience and the capacity to handle crises become part of the hospitals' organizational culture. To become more resilient to crises, a raised awareness--especially in the area of human resource (HR)--is necessary. The aim of this paper is to contribute to the process robustness against crises through the identification and evaluation of relevant HR crises and their causations in hospitals. Qualitative and quantitative methods were combined to identify and evaluate crises in hospitals in the HR sector. A structured workshop with experts was conducted to identify HR crises and their descriptions, as well as causes and consequences for patients and hospitals. To evaluate the findings, an online survey was carried out to rate the occurrence (past, future) and dangerousness of each crisis. Six HR crises were identified in this study: staff shortages, acute loss of personnel following a pandemic, damage to reputation, insufficient communication during restructuring, bullying, and misuse of drugs. The highest occurrence probability in the future was seen in staff shortages, followed by acute loss of personnel following a pandemic. Staff shortages, damage to reputation, and acute loss of personnel following a pandemic were seen as the most dangerous crises. The study concludes that coping with HR crises in hospitals is existential for hospitals and requires increased awareness. The six HR crises identified occurred regularly in German hospitals in the past, and their occurrence probability for the future was rated as high.
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Fludarabine Based Conditioning for Allogeneic Transplantation for Advanced Hematologic Malignancies
2017-10-25
Acute Myeloid Leukemia; Acute Leukemia; Chronic Myelogenous Leukemia; Malignant Lymphoma; Hodgkin's Disease; Multiple Myeloma; Lymphocytic Leukemia; Myeloproliferative Disorder; Polycythemia Vera; Myelofibrosis; Aplastic Anemia
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T-Cell Depleted Allogeneic Stem Cell Transplantation for Patients With Hematologic Malignancies
2016-10-07
Acute Myelogenous Leukemia; Lymphoid Leukemia; Chronic Myelogenous Leukemia; Malignant Lymphoma; Hodgkin's Disease; Chronic Lymphocytic Leukemia; Myeloproliferative Disorder; Anemia, Aplastic; Myelodysplastic Syndromes
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... inherited Pregnancy Problems with bone marrow such as lymphoma, leukemia, myelodysplasia, multiple myeloma, or aplastic anemia Slow blood loss (for example, from heavy menstrual periods or stomach ulcers ) Sudden heavy blood loss
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Methemoglobinemia in Young Patients With Hematologic Cancer or Aplastic Anemia Treated With Dapsone
2017-04-13
Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Methemoglobinemia; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Nonmalignant Neoplasm
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Unrelated Umbilical Cord Blood (UBC)Transplantation
2018-05-25
Chronic Myelogenous Leukemia (CML); Acute Myelogenous Leukemia (AML); Myelodysplastic Syndrome; Multiple Myeloma; Hodgkin Lymphoma; Non-Hodgkin Lymphoma; Chronic Lymphocytic Leukemia (CLL); Acute Lymphocytic Leukemia (ALL); Severe Aplastic Anemia
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Acquired factor VII deficiency associated with acute myeloid leukemia.
Anoun, Soumaya; Lamchahab, Mouna; Oukkache, Bouchra; Qachouh, Maryam; Benchekroun, Said; Quessar, Asmaa
2015-04-01
Isolated acquired factor VII deficiency is a rare coagulopathy. It has been reported in 31 patients with malignancy, sepsis, postoperatively, aplastic anemia, and during bone marrow transplantation. We discuss, through a new case of acquired factor VII deficiency, the characteristics of this disease when it is associated with acute myeloid leukemia. Acquired factor VII deficiency in hematological diseases can be caused by intensive chemotherapy, infections, or hepatic dysfunction. The best treatment in developing countries remains corticosteroids associated with plasma exchange, frozen plasma, and antibiotics.
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Resilience of Natural Gas Networks during Conflicts, Crises and Disruptions
Carvalho, Rui; Buzna, Lubos; Bono, Flavio; Masera, Marcelo; Arrowsmith, David K.; Helbing, Dirk
2014-01-01
Human conflict, geopolitical crises, terrorist attacks, and natural disasters can turn large parts of energy distribution networks offline. Europe's current gas supply network is largely dependent on deliveries from Russia and North Africa, creating vulnerabilities to social and political instabilities. During crises, less delivery may mean greater congestion, as the pipeline network is used in ways it has not been designed for. Given the importance of the security of natural gas supply, we develop a model to handle network congestion on various geographical scales. We offer a resilient response strategy to energy shortages and quantify its effectiveness for a variety of relevant scenarios. In essence, Europe's gas supply can be made robust even to major supply disruptions, if a fair distribution strategy is applied. PMID:24621655
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Ledgerd, Ritchard; Hoe, Juanita; Hoare, Zoë; Devine, Mike; Toot, Sandeep; Challis, David; Orrell, Martin
2016-06-01
Crisis situations in dementia can lead to hospital admission or institutionalisation. Offering immediate interventions may help avoid admission, whilst stabilising measures can help prevent future crises. Our objective was to identify the main causes of crisis and interventions to treat or prevent crisis in persons with dementia based on different stakeholder perspectives. An online questionnaire was developed to identify the causes of crisis and appropriate interventions in a crisis. Participants included people with dementia, family carers and staff working in health and social care, including emergency and voluntary sectors, and academia. The results ranked the main causes of crisis, interventions that can prevent a crisis and interventions that can be useful in a crisis. Wandering, falls and infection were highly rated as risk factors for crises across all stakeholder groups. Consumers rated aggression as less important but severity of memory impairment as much more important than the other groups did. Education and support for family carers and home care staff were highly valued for preventing crises. Well-trained home care staff, communication equipment, emergency contacts and access to respite were highly valued for managing crises. We identified triggers and interventions that different stakeholders see as important for crisis in dementia. Recognition of these may be critical to planning effective and accepted support and care for people with dementia. Copyright © 2015 John Wiley & Sons, Ltd.
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Wang, Xin; Ma, Feng-Xia; Lu, Shi-Hong; Chi, Ying; Chen, Fang; Li, Xue; Li, Juan-Juan; Du, Wen-Jing; Feng, Ying; Cui, Jun-Jie; Song, Bao-Quan; Han, Zhong-Chao
2014-06-01
This study was aimed to investigate the effects of rapamycin on biological function and autophagy of bone marrow mesenchymal stem cells (BM-MSC) from patients with aplastic anemia so as to provide experimental basis for the clinical treatment of aplastic anemia (AA) with rapamycin. BM-MSC were treated with different concentrations of rapamycin (0, 10, 50, 100 nmol/L) for 48 h, the expression of LC3B protein was detected by Western blot to observe the effect of rapamycin on cell autophagy; cell apoptosis and cell cycles were detected by flow cytometry; the proliferation of BM-MSC of AA patients was measured by cell counting kit-8; the adipogenic differentiation of BM-MSC were tested by oil red O staining after adipogenic induction for 2 weeks; the adipogenic related genes (LPL, CFD, PPARγ) were detected by real-time PCR. The results showed that the proliferation and adipogenesis of BM-MSC of AA patients were inhibited by rapamycin. Moreover, the autophagy and apoptosis of BM-MSC were increased by rapamycin in a dose-dependent way.Rapamycin arrested the BM-MSC in G0/G1 phase and prevented them into S phase (P < 0.05). It is concluded that rapamycin plays an critical role in inhibiting cell proliferation, cell cycles, and adipogenesis, these effects may be related with the autophagy activation and mTOR inhibition resulting from rapamycin.
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Marsh, Judith C; Bacigalupo, Andrea; Schrezenmeier, Hubert; Tichelli, Andre; Risitano, Antonio M; Passweg, Jakob R; Killick, Sally B; Warren, Alan J; Foukaneli, Theodora; Aljurf, Mahmoud; Al-Zahrani, H A; Höchsmann, Britta; Schafhausen, Philip; Roth, Alexander; Franzke, Anke; Brummendorf, Tim H; Dufour, Carlo; Oneto, Rosi; Sedgwick, Philip; Barrois, Alain; Kordasti, Shahram; Elebute, Modupe O; Mufti, Ghulam J; Socie, Gerard
2012-06-07
Rabbit antithymocyte globulin (rATG; thymoglobulin, Genzyme) in combination with cyclosporine, as first-line immunosuppressive therapy, was evaluated prospectively in a multicenter, European, phase 2 pilot study, in 35 patients with aplastic anemia. Results were compared with 105 age- and disease severity-matched patients from the European Blood and Marrow Transplant registry, treated with horse ATG (hATG; lymphoglobulin) and cyclosporine. The primary end point was response at 6 months. At 3 months, no patients had achieved a complete response to rATG. Partial response occurred in 11 (34%). At 6 months, complete response rate was 3% and partial response rate 37%. There were 10 deaths after rATG (28.5%) and 1 after subsequent HSCT. Infections were the main cause of death in 9 of 10 patients. The best response rate was 60% for rATG and 67% for hATG. For rATG, overall survival at 2 years was 68%, compared with 86% for hATG (P = .009). Transplant-free survival was 52% for rATG and 76% for hATG (P = .002). On multivariate analysis, rATG (hazard ratio = 3.9, P = .003) and age more than 37 years (hazard ratio = 4.7, P = .0008) were independent adverse risk factors for survival. This study was registered at www.clinicaltrials.gov as NCT00471848.
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Gupta, Ashish; Fu, Pingfu; Hashem, Hasan; Vatsayan, Anant; Shein, Steven; Dalal, Jignesh
2017-12-01
Aplastic anemia is a bone marrow failure syndrome with high mortality affecting children and young adults. Although current treatment guidelines recommend hematopoietic stem cell transplant (HCT) for patients with matched sibling donors, outcomes with alternate donor options have been improving. We analyzed a validated multiinstitutional pediatric cohort using one of the largest pediatric and young adult database, the Pediatric Health Information System, for patients diagnosed with aplastic anemia (AA) from 2006 to 2015. Outcomes with upfront and salvage transplants were analyzed along with healthcare utilization. Among 2,169 patients in the study period, almost 20% underwent HCT, while others received immunosuppressive therapy. In a multivariate model, there was no significant difference in mortality with upfront or salvage transplants (odds ratio [OR] 1.24, 95% confidence interval [CI] 0.6-2.58, P = 0.567), while every platelet transfusion was associated with higher mortality (OR 1.37, 95% CI 1.12-1.67, P = 0.002). Healthcare utilization was significantly higher in salvage transplants requiring frequent hospitalization and transfusion requirements. Treatment mortality and graft failure rates were significantly reduced in the salvage transplant group in recent years (2011-2015 as compared to 2006-2010). As outcomes with HCT continue to improve in severe AA, transplant with good alternate donors should be considered upfront in children and young adults. © 2017 Wiley Periodicals, Inc.
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[Womanhood today--identity experiences and identity crises].
Kast, V
1985-01-01
Modern women's identity crises and the various possibilities of identification along the way towards a new identity can be seen as her attempts to develop out of the depressive situation that her once normal role identity had, to a large extent, placed her in. Under this aspect, even concepts of living that are seen by many to be problematic can be justified as leading along the way towards identity, which is so essential for human relationships and interpersonal empathy.
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Myers, Risa B; Lazaridis, Christos; Jermaine, Christopher M; Robertson, Claudia S; Rusin, Craig G
2016-09-01
To develop computer algorithms that can recognize physiologic patterns in traumatic brain injury patients that occur in advance of intracranial pressure and partial brain tissue oxygenation crises. The automated early detection of crisis precursors can provide clinicians with time to intervene in order to prevent or mitigate secondary brain injury. A retrospective study was conducted from prospectively collected physiologic data. intracranial pressure, and partial brain tissue oxygenation crisis events were defined as intracranial pressure of greater than or equal to 20 mm Hg lasting at least 15 minutes and partial brain tissue oxygenation value of less than 10 mm Hg for at least 10 minutes, respectively. The physiologic data preceding each crisis event were used to identify precursors associated with crisis onset. Multivariate classification models were applied to recorded data in 30-minute epochs of time to predict crises between 15 and 360 minutes in the future. The neurosurgical unit of Ben Taub Hospital (Houston, TX). Our cohort consisted of 817 subjects with severe traumatic brain injury. Our algorithm can predict the onset of intracranial pressure crises with 30-minute advance warning with an area under the receiver operating characteristic curve of 0.86 using only intracranial pressure measurements and time since last crisis. An analogous algorithm can predict the start of partial brain tissue oxygenation crises with 30-minute advanced warning with an area under the receiver operating characteristic curve of 0.91. Our algorithms provide accurate and timely predictions of intracranial hypertension and tissue hypoxia crises in patients with severe traumatic brain injury. Almost all of the information needed to predict the onset of these events is contained within the signal of interest and the time since last crisis.
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Vulnerability of countries to food-production crises propagating in the virtual water trade network
NASA Astrophysics Data System (ADS)
Tamea, S.; Laio, F.; Ridolfi, L.
2015-12-01
In recent years, the international trade of food and agricultural commodities has undergone a marked increase of exchanged volumes and an expansion of the trade network. This globalization of trade has both positive and negative effects, but the interconnectedness and external dependency of countries generate complex dynamics which are often difficult to understand and model. In this study we consider the volume of water used for the production of agricultural commodities, virtually exchanged among countries through commodity trade, i.e. the virtual water trade. Then, we set up a parsimonious mechanistic model describing the propagation, into the global trade network, of food-production crises generated locally by a social, economic or environmental event (such as war, economic crisis, drought, pest). The model, accounting for the network structure and the virtual water balance of all countries, bases on rules derived from observed virtual water flows and on data-based and statistically verified assumption. It is also tested on real case studies that prove its capability to capture the main features of crises propagation. The model is then employed as the basis for the development of an index of country vulnerability, measuring the exposure of countries to crises propagating in the virtual water trade network. Results of the analysis are discussed within the context of socio-economic and environmental conditions of countries, showing that not only water-scarce, but also wealthy and globalized countries, are among the most vulnerable to external crises. The temporal analysis for the period 1986-2011 reveals that the global average vulnerability has strongly increased over time, confirming the increased exposure of countries to external crises which may occur in the virtual water trade network.
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Bone Marrow Transplantation of Patients in Remission Using Partially Matched Relative Donor
2016-10-19
Acute Myeloid Leukemia; Myelodysplastic Syndromes; Biphenotypic Leukemia; Acute Lymphocytic Leukemia; Chronic Myeloid Leukemia; Chronic Lymphocytic Leukemia; Plasma Cell Neoplasms; Lymphoma; Hodgkin's Disease; Aplastic Anemia
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Immunosuppressive therapy for patients with Down syndrome and idiopathic aplastic anemia.
Suzuki, Kyogo; Muramatsu, Hideki; Okuno, Yusuke; Narita, Atsushi; Hama, Asahito; Takahashi, Yoshiyuki; Yoshida, Makoto; Horikoshi, Yasuo; Watanabe, Ken-Ichiro; Kudo, Kazuko; Kojima, Seiji
2016-07-01
Idiopathic aplastic anemia (AA) is a rare hematological complication of Down syndrome (DS). The safety and efficacy of immunosuppressive therapy (IST) in individuals with DS remain unknown. We used a standard regimen of IST, comprising antithymocyte globulin and cyclosporine A, to treat three children with DS and idiopathic acquired AA. Two patients achieved a hematological (complete or partial) response and became transfusion independent at the final follow-up. The third patient failed to respond to IST and underwent bone marrow transplantation from a human leukocyte antigen (HLA)-mismatched unrelated donor. None of the patients experienced severe or unexpected adverse events during IST. Our experience suggests that IST is a safe and reasonable treatment, even in individuals with DS who suffer from AA and lack an HLA-matched sibling donor.
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Novotný, J; Zvarová, M; Prazáková, L; Jandlová, M; Konvicková, L
1995-10-01
Aplastic anaemia (AA) of the chronic type with severe cytopenia is very frequently a difficult therapeutic problem. Patients with granulocyte values below 0.5 G/l are threatened by infections, incl. sepsis possibly with a fatal outcome. If the pool of stem cells for granulocytes is not completely exhausted and can respond to growth factors, these patients can be treated either chronically and/or in risk situations (e.g. injury, surgery) with preparations of the type of a recombinant, granulocyte colony stimulating factor (rhG-CSF), or granulocyte and monocyte colony stimulating factor (rhGM-CSF). The authors present a review of diagnostic and therapeutic algorithms in patients with the AA syndrome and summarize their own experience with the preparation Neupogen Roche (rhG-CSF).
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Tachi, N.; Shimotori, S.; Naruse, N.
Synthetic adhesives are widely used in various industries as well as at home. Adhesives usually contain several organic solvents which easily vaporize. Exposure can cause aplastic anemia and polyneuropathy in adults. Chronic glue sniffing results in aplastic anemia, polyneuropathy, and muscular atrophy. Inhalation of the solvent contained in adhesives, such as n-hexane, toluene, xylene, and benzene by pregnant animals can decrease the number of live fetuses and retard fetal growth. In humans, the risk of spontaneous abortion is increased in workers exposed to organic solvents. However, information is still limited about the effects of exposure to organic solvents vaporized frommore » adhesives on fetuses. In the present study, female mice were exposed throughout pregnancy to organic solvents vaporized from an adhesive to clarify the effects of the inhalation on progeny. 19 refs., 1 fig., 4 tabs.« less
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Zeidan, Amer M; Battiwalla, Minoo; Berlyne, Deborah; Winkler, Thomas
2017-02-01
Patients with acquired and inherited bone marrow failure syndromes (BMFS) have ineffective hematopoiesis due to impairments of the hematopoietic stem cell compartment. Common manifestations of BMFS include varying degrees of peripheral blood cytopenias and, sometimes, progression to acute myelogenous leukemia. Research efforts have been made all over the world to improve understanding of the pathogenesis of these diseases and their clinical implications. The Aplastic Anemia and MDS International Foundation (AAMDSIF) is an independent nonprofit organization whose mission is to help patients and family members cope with BMFS. Here, we summarize recent scientific discoveries in several BMFS that were presented at the fifth International Bone Marrow Failure Disease Scientific Symposium 2016 that AAMDSIF sponsored on March 17-18, 2016, in Rockville, Maryland. Copyright © 2016 Elsevier Ltd. All rights reserved.
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Bronn, L.J.; Paquelet, J.R.; Tetalman, M.R.
Imaging of the bone marrow by radionuclide scanning was performed using colloids, which are phagocytized by the reticuloendothelial cells of the marrow, or radioiron, which is incorporated into reticulocytes. The use of the former radiopharmaceutical is based on the assumption, generally valid except in aplastic states or after irradiation, that the distribution of hematopoietic and reticuloendothelial tissue in the marrow is similar. Regardless of the method used, active adult marrow is normally distributed only in the axial skeleton and proximal humeri and femurs. Marrow imaging has been used in the evaluation of myeloproliferative disorders, leukemia, lymphoma, aplastic states, malignancy metastaticmore » to marrow, and hemolytic anemia. We report a case of thalassemia major in which the diagnosis of intrathoracic extramedullary hematopoiesis was confirmed with the /sup 99m/Tc sulfur colloid bone marrow scan.« less
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A composite mouse model of aplastic anemia complicated with iron overload
Wu, Dijiong; Wen, Xiaowen; Liu, Wenbin; Xu, Linlong; Ye, Baodong; Zhou, Yuhong
2018-01-01
Iron overload is commonly encountered during the course of aplastic anemia (AA), but no composite animal model has been developed yet, which hinders drug research. In the present study, the optimal dosage and duration of intraperitoneal iron dextran injection for the development of an iron overload model in mice were explored. A composite model of AA was successfully established on the principle of immune-mediated bone marrow failure. Liver volume, peripheral hemogram, bone marrow pathology, serum iron, serum ferritin, pathological iron deposition in multiple organs (liver, bone marrow, spleen), liver hepcidin, and bone morphogenetic protein 6 (BMP6), SMAD family member 4 (SMAD4) and transferrin receptor 2 (TfR2) mRNA expression levels were compared among the normal control, AA, iron overload and composite model groups to validate the composite model, and explore the pathogenesis and features of iron overload in this model. The results indicated marked increases in iron deposits, with significantly increased liver/body weight ratios as well as serum iron and ferritin in the iron overload and composite model groups as compared with the normal control and AA groups (P<0.05). There were marked abnormalities in iron regulation gene expression between the AA and composite model groups, as seen by the significant decrease of hepcidin expression in the liver (P<0.01) that paralleled the changes in BMP6, SMAD4, and TfR2. In summary, a composite mouse model with iron overload and AA was successfully established, and AA was indicated to possibly have a critical role in abnormal iron metabolism, which promoted the development of iron deposits. PMID:29434729
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A composite mouse model of aplastic anemia complicated with iron overload.
Wu, Dijiong; Wen, Xiaowen; Liu, Wenbin; Xu, Linlong; Ye, Baodong; Zhou, Yuhong
2018-02-01
Iron overload is commonly encountered during the course of aplastic anemia (AA), but no composite animal model has been developed yet, which hinders drug research. In the present study, the optimal dosage and duration of intraperitoneal iron dextran injection for the development of an iron overload model in mice were explored. A composite model of AA was successfully established on the principle of immune-mediated bone marrow failure. Liver volume, peripheral hemogram, bone marrow pathology, serum iron, serum ferritin, pathological iron deposition in multiple organs (liver, bone marrow, spleen), liver hepcidin, and bone morphogenetic protein 6 (BMP6), SMAD family member 4 (SMAD4) and transferrin receptor 2 (TfR2) mRNA expression levels were compared among the normal control, AA, iron overload and composite model groups to validate the composite model, and explore the pathogenesis and features of iron overload in this model. The results indicated marked increases in iron deposits, with significantly increased liver/body weight ratios as well as serum iron and ferritin in the iron overload and composite model groups as compared with the normal control and AA groups (P<0.05). There were marked abnormalities in iron regulation gene expression between the AA and composite model groups, as seen by the significant decrease of hepcidin expression in the liver (P<0.01) that paralleled the changes in BMP6, SMAD4, and TfR2. In summary, a composite mouse model with iron overload and AA was successfully established, and AA was indicated to possibly have a critical role in abnormal iron metabolism, which promoted the development of iron deposits.
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Nonmyeloablative Allo SCT for the Treatment of Hematologic Disorders
2017-04-05
AML; ALL; CML Chronic Phase, Accelerated Phase, or Blast Crisis; CLL; MDS; RELAPSED NON-HODGKIN'S OR HODGKIN'S LYMPHOMA; APLASTIC ANEMIA; MULTIPLE MYELOMA; MYELOPROLIFERATIVE DISORDER (P Vera, CMML, ET)
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Skip to main content U.S. Department of Health & Human Services Health Topics Health Topics A-Z Clinical Trials Publications and Resources Health Education and Awareness The Science Science Home Blood Disorders and ...
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Keohane, Elaine M
2004-01-01
Acquired aplastic anemia (AA) is a disorder characterized by a profound deficit of hematopoietic stem and progenitor cells, bone marrow hypocellularity, and peripheral blood pancytopenia. It primarily affects children, young adults, and those over 60 years of age. The majority of cases are idiopathic; however, idiosyncratic reactions to some drugs, chemicals, and viruses have been implicated in its etiology. An autoimmune T-cell reaction likely causes the stem cell depletion, but the precise mechanism, as well as the eliciting and target antigens, is unknown. Symptoms vary from severe life-threatening cytopenias to moderate or non-severe disease that does not require transfusion support. The peripheral blood typically exhibits pancytopenia, reticulocytopenia, and normocytic or macrocytic erythrocytes. The bone marrow is hypocellular and may exhibit dysplasia of the erythrocyte precursors. First line treatment for severe AA consists of hematopoietic stem cell transplantation in young patients with HLA identical siblings, while immunosuppression therapy is used for older patients and for those of any age who lack a HLA matched donor. Patients with AA have an increased risk of developing paroxysmal nocturnal hemoglobinuria (PNH), myelodysplastic syndrome (MDS), or acute leukemia. Further elucidation of the pathophysiology of this disease will result in a better understanding of the interrelationship among AA, PNH, and MDS, and may lead to novel targeted therapies.
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[A Case of Severe Chronic Active Epstein-Barr Virus Infection with Aplastic Anemia and Hepatitis].
Lee, Ja In; Lee, Sung Won; Han, Nam Ik; Ro, Sang Mi; Noh, Yong-Sun; Jang, Jeong Won; Bae, Si Hyun; Choi, Jong Young; Yoon, Seung Kew
2016-01-25
Epstein-Barr virus (EBV) causes various acute and chronic diseases. Chronic active EBV infection (CAEBV) is characterized by infectious mononucleosis-like symptoms that persist for more than 6 months with high viral loads in peripheral blood and/or an unusual pattern of anti-EBV antibodies. Severe CAEBV is associated with poor prognosis with severe symptoms, an extremely high EBV-related antibody titer, and hematologic complications that often include hemophagocytic lymphohistiocytosis. However, CAEBV which led to the development of aplastic anemia (AA) has not been reported yet. A 73-year-old woman was admitted to our hospital with intermittent fever, general weakness and elevated liver enzymes. In the serologic test, EBV-related antibody titer was elevated, and real-time quantitative-PCR in peripheral blood showed viral loads exceeding 10(4) copies/μg DNA. Liver biopsy showed characteristic histopathological changes of EBV hepatitis and in situ hybridization with EBV-encoded RNA-1 was positive for EBV. Pancytopenia was detected in peripheral blood, and the bone marrow aspiration biopsy showed hypocellularity with replacement by adipocytes. AA progressed and the patient was treated with prednisolone but deceased 8 months after the diagnosis due to multiple organ failure and opportunistic infection. Herein, we report a rare case of severe CAEBV in an adult patient accompanied by AA and persistent hepatitis.
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Chen, Miao; Zhuang, Junling; Zhou, Daobin; Xu, Ying; Zhao, Yongqiang; Wang, Shujie; Zhang, Wei; Duan, Minghui; Zhu, Tienan; Li, Jian; Cai, Huacong; Cao, Xinxin; Han, Bing
2017-04-01
The influence of chronic hepatitis B virus (HBV) infection on the efficacy of intensive immunosuppressive treatment (IST) of severe aplastic anaemia (SAA) patients remains unclear. Previous reports on this topic have been mostly case reports or have had a relatively short follow-up. Eight SAA patients carrying chronic HBV infection and 24 matched patients without HBV at a ratio of 1:3 were included in this retrospective analysis. The patients were treated with anti-thymocyte globulin (ATG) and cyclosporine A. Entecavir was or was not administered throughout the IST course to patients with positive or negative HBV-DNA results, respectively. No evident HBV reactivation developed. The overall response was 87.5% by 12 months, and the recurrence rate was 12.5%. There were no significant differences in overall response, overall survival and event-free survival between groups. Entecavir can effectively prevent reactivation of HBV in SAA patients with positive HBV-DNA who received intensive IST. Regular surveillance may be sufficient for HBV-DNA negative patients who should receive antiviral drugs immediately when their HBV-DNA status changes from negative to positive. The prognosis of SAA patients with chronic HBV infection after intensive IST treatment is not worse than those without HBV infection.
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Review of book vestibular crises
NASA Technical Reports Server (NTRS)
Blagoveshchenskaya, N. S.
1980-01-01
The etiology, pathogenesis, clinical practice, treatment and rehabilitation of patients with vestibular crises is discussed. Classifications for vestibular disorders are given. Information on the frequency of vestibular crises is given.
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[Adolescent crises in puberty. Diagnosis and therapy (author's transl)].
Müller-Küppers, M
1979-08-24
Psychological disorders which become manifest as adolescent crises must be seen as complex phenomena and treated thoroughly: besides the genetic, biographic and psychosocial background of the youthful individual the cultural and economic aspects play an important role. Nevertheless we must admit that associated causal explanations, e.g. for accelerated or late development are lacking. Focal points for the subsequent symptom complexes of sexual behavior in puberty are: psychosexual prematurity or retardation, masturbation, homosexual relations, pubertal asceticism and premature and frequently changing sexual relations.
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... del paciente Transplant process Diseases treated by transplant Acute myeloid leukemia Adrenoleukodystrophy (ALD) Chronic Lymphocytic Leukemia (CLL) ... SCID) Sickle cell disease (SCD) Wiskott-Aldrich syndrome Acute lymphoblastic leukemia (ALL) Other diseases Treatment decisions Learn ...
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The role of parvovirus B19 and the immune response in the pathogenesis of acute leukemia.
Kerr, Jonathan R; Mattey, Derek L
2015-05-01
In this article, we review the evidence suggesting a possible role for B19 virus in the pathogenesis of a subset of cases of acute leukemia. Human parvovirus B19 infection may complicate the clinical course of patients with acute leukemia and may also precede the development of acute leukemia by up to 180 days. Parvovirus B19 targets erythroblasts in the bone marrow and may cause aplastic crisis in patients with shortened-red cell survival. Aplastic crisis represents a prodrome of acute lymphoblastic leukemia in 2% patients. There is a significant overlap between those HLA classes I and II alleles that are associated with a vigorous immune response and development of symptoms during B19 infection and those HLA alleles that predispose to development of acute leukemia. Acute symptomatic B19 infection is associated with low circulating IL-10 consistent with a vigorous immune response; deficient IL-10 production at birth was recently found to be associated with subsequent development of acute leukemia. Anti-B19 IgG has been associated with a particular profile of methylation of human cancer genes in patients with acute leukemia, suggesting an additional hit and run mechanism. The proposed role for parvovirus B19 in the pathogenesis of acute leukemia fits well with the delayed infection hypothesis and with the two-step mutation model, which describes carriage of the first mutation prior to birth, followed by suppression of hematopoiesis, which allows rapid proliferation of cells harboring the first mutation, acquisition of a second activating mutation, and expansion of cells carrying both mutations, resulting in acute leukemia. Copyright © 2015 John Wiley & Sons, Ltd.
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Crawford, Doreen; Dearmun, Annette
2017-02-06
Fanconi anaemia is a rare inherited genetic condition that can lead to aplastic anaemia and bone marrow failure. People with the condition have a predisposition to some cancers. The condition is named after Swiss paediatrician Guido Fanconi.
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Aplastic Anemia and Myelodysplastic Syndromes
Skip to main content U.S. Department of Health and Human Services Follow us: Search Menu Search for Information from NIDDK Entire Site Research & Funding Health Information About NIDDK News Search Research & Funding Current ...
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2009-01-22
Chronic Myeloid Leukemia; Acute Myelogenous Leukemia; Myelodysplasia; Acute Lymphocytic Leukemia; Severe Aplastic Anemia; Non-Hodgkin's Lymphoma; Lymphoproliferative Disease; Multiple Myeloma; Advanced Myeloproliferative Disease
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Unrelated Donor Stem Cell Transplantation
2013-12-05
Severe Aplastic Anemia; Paroxysmal Nocturnal Hemoglobinuria; Acute Myelogenous Leukemia; Acute Lymphoblastic Leukemia; Myelodysplastic Syndromes; Myeloproliferative Syndromes; Chronic Myelogenous Leukemia; Hodgkin's Lymphoma; Non-Hodgkin's Lymphoma; Multiple Myeloma; Chronic Lymphocytic Leukemia; Small Lymphocytic Lymphoma; Large Granulocytic Leukemia
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2018-01-30
Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Diseases; Nonmalignant Neoplasm; Unspecified Adult Solid Tumor, Protocol Specific; Unspecified Childhood Solid Tumor, Protocol Specific
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Alefacept and Allogeneic Hematopoietic Stem Cell Transplantation
2017-07-24
Thalassemia; Sickle Cell Disease; Glanzmann Thrombasthenia; Wiskott-Aldrich Syndrome; Chronic-granulomatous Disease; Severe Congenital Neutropenia; Leukocyte Adhesion Deficiency; Schwachman-Diamond Syndrome; Diamond-Blackfan Anemia; Fanconi Anemia; Dyskeratosis-congenita; Chediak-Higashi Syndrome; Severe Aplastic Anemia
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Kobayashi, Ryoji; Yabe, Hiromasa; Hara, Junichi; Morimoto, Akira; Tsuchida, Masahiro; Mugishima, Hideo; Ohara, Akira; Tsukimoto, Ichiro; Kato, Koji; Kigasawa, Hisato; Tabuchi, Ken; Nakahata, Tatsutoshi; Ohga, Shoichi; Kojima, Seiji
2006-12-01
The incidence of graft rejection was determined in 66 children with acquired aplastic anaemia (AA) following bone marrow transplantation (BMT) from a related donor. Eleven of 65 evaluable patients experienced either early or late rejection. Multivariate analysis identified previous immunosuppressive therapy with antithymocyte-globulin (ATG) and ciclosporin (CsA) as a risk factor for graft rejection (relative risk: 16.6, P = 0.001). Patients who received ATG and CsA had a significantly lower probability of failure-free survival than those who did not (69.7 +/- 6.2% vs. 87.9 +/- 8.0%, P = 0.044). These results suggest that BMT should be instituted immediately in children with severe AA who have human leucocyte antigen-identical siblings.
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Luo, Cheng-Juan; Gao, Yi-Jin; Tang, Jing-Yan; Zhu, Xiao-Hua; Xue, Hui-Liang; Lu, Feng-Juan; Pan, Ci; Jiang, Hua; Luo, Chang-Ying; Ye, Qi-Dong; Zhou, Min; Chen, Jing
2014-07-01
For the first time, we conducted a 2-center retrospective study to show the efficacy of antithymocyte globulin (ATG)-Fresenius S plus cyclosporine treatment of children with severe aplastic anemia. From March 1997 to May 2011, a total of 124 patients (median age, 7.5 y; range, 1.5 to 16 y) from 2 centers with acquired AA treated with an immunosuppressive therapy (IST) regimen, consisting of ATG-Fresenius S (5 mg/kg per day for 5 d) and cyclosporine, were enrolled. The response rate was 55.6%. The median time between IST and response was 6 (0.5 to 18) months. After a median follow-up time of 29 (6 to 153) months, the rates of relapse and clonal evolution were 3.2% and 0.8%, respectively. Overall, 17 patients (13.7%) died in this study: 14 resulted from sepsis, 1 resulted from intracranial hemorrhage, 1 occurred after hematopoietic stem cell transplantation, and 1 resulted from clonal disease progression. The 5-year overall survival rate for the entire cohort was 74.7%. IST responders had a better survival rate (100%) than nonresponders (70.7%). The use of ATG-Fresenius S plus cyclosporine as a first-line immunosuppressive treatment appeared to be effective for children with severe aplastic anemia in our study. ATG-Fresenius S could be another option in the treatment arsenal, especially in countries where the other ATG products are harder to acquire.
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[Medication-related oculogyric crises: a description of four cases and a review of the literature].
Darling, A; Poo, P; Perez-Duenas, B; Campistol, J
2013-02-01
Oculogyric crises are considered to be a form of focal dystonia and can be observed as reactions to pharmaceuticals. The signs and symptoms may be confused with epileptic crises. To describe the clinical features and progress of patients with pharmaceutical-related oculogyric crises and to carry out a review of the topic. We conducted a retrospective, descriptive study of four patients evaluated in the neurology service due to oculogyric crises. The patients had been diagnosed with an associated conduct disorder requiring treatment with antipsychotic drugs. The episodes of oculogyric crises did not correlate with the findings in the electroencephalogram. They responded well to the reduction in dosage or to withdrawal of the apparent causing agent. The clinical picture does not present only in patients treated with antipsychotics but is also linked with other pharmaceuticals that are frequently used in daily paediatric practice. When oculogyric crises are the reason for visiting, differential diagnoses must be taken into account in order to avoid unnecessary studies and to carry out an appropriate therapeutic management.
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Hepatitis A and parvovirus B19 infections in an infant with fulminant hepatic failure.
Ozçay, Figen; Bikmaz, Y Emre; Canan, Oğuz; Ozbek, Namik
2006-06-01
Acute viral hepatitis with hepatitis A, B, C, D, and E viruses in the etiology of fulminant hepatic failure either single or in combinations has been described. Parvovirus B19 is also an etiologic agent of acute liver failure and hepatitis-associated aplastic anemia. We present a patient diagnosed with fulminant hepatitis A referred for liver transplantation. Parvovirus B19 superinfection was detected when the patient developed anemia during the course of the disease. We discuss possible roles of both viruses in fulminant hepatitis and pure red cell aplasia.
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Bone-marrow transplant - series (image)
Bone-marrow transplants are performed for: deficiencies in red blood cells (aplastic anemia) and white blood cells (leukemia or ... Bone-marrow transplants prolong the life of patients who might otherwise die. As with all major organ transplants, however, ...
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Health crises and media relations: relationship management-by-fire.
Springston, Jeffrey K; Weaver-Lariscy, Ruthann
2007-01-01
Media relations is an important function in the operation of any health organization, yet it is often relegated as a simple task function. Such an orientation can be problematic, particularly in times of crisis. This article provides an overview of some of the inherent internal conflicts within health organizations that may mitigate against the best media relations practices in times of crises. The article surveys some of the predominant theoretical models used for crisis management, and suggests directions for the further development of media relations and crisis communication theory and practice.
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ERIC Educational Resources Information Center
Caudle, Melissa
1994-01-01
School crises may be categorized as emergency situations, human-made crises, natural events, medical emergencies, and mechanical crises. Central to any successful crisis-management plan are onsite and district-level crisis response teams. Plans should specify staff responsibilities; provide for communication codes, devices, and procedures;…
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Investigation on financial crises with the negative-information-propagation-induced model
NASA Astrophysics Data System (ADS)
Fan, Feng-Hua; Deng, Yanbin; Huang, Yong-Chang
2017-03-01
We first argue about the similarity between the propagation phenomenon of negative information about potential deterioration of economic situation in group of investors and the propagation phenomenon of infectious disease in crowd Applying the negative-information-propagation-induced model built based on above argument, we investigate the relationship between the generation of financial crises and propagation effects of negative information We introduce the discrimination parameter to distinguish whether or not negative information will be propagated extensively in group of investors. We also introduce the target critical value of financial crises. By comparing the theoretically predicted ratio of the long term projected number of total investors to the total number of investors at some time as initial time with target critical value of financial crises, the model can provide real-time monitoring of whether the curve of total number of investors is progressing toward the direction of generating financial crises or running on track of financial markets safety. If at some time this ratio is computed to be less than the target critical value of financial crises, governments can take relevant measures to prevent the generation of financial crises in advance Governments' interference helps to recover the confidence of investors so that they never will again believe in negative information to continue their investment. Results from theoretical and numerical analysis show that the number of investors who hold the belief of potential deterioration of economic situation, and the number of investors who withdraw capital and depart from financial markets for avoiding business loss when governments make appropriate interference are lowered compared to that without appropriate governments' interference. The results show the effectiveness of governments in preventing financial crises from the viewpoint of the negative information-propagation-induced model, namely governments' prevention against financial crises can reduce the possibility of the generation of financial crises.
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Nonmyeloablative Allogeneic Transplant
2013-12-05
Aplastic Anemia; Paroxysmal Nocturnal Hemoglobinuria; Acute Myelogenous Leukemia; Acute Lymphocytic Leukemia; Myelodysplastic Syndrome; Chronic Myelogenous Leukemia; Chronic Lymphocytic Leukemia; Hodgkin's Lymphoma; Non-Hodgkin's Lymphoma; Mantle Cell Lymphoma; Multiple Myeloma; Waldenstrom Macroglobulinemia; Breast Cancer; Renal Cell Carcinoma; Melanoma; Sarcoma; Ovarian Cancer; Thymoma
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A new NCI study has found that, among patients with severe aplastic anemia who received a hematopoietic cell transplant from an unrelated donor, those whose donor white blood cells had longer telomeres had higher survival rates five-years after transplant
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BMT Abatacept for Non-Malignant Diseases
2018-05-16
Hurler Syndrome; Fanconi Anemia; Glanzmann Thrombasthenia; Wiskott-Aldrich Syndrome; Chronic Granulomatous Disease; Severe Congenital Neutropenia; Leukocyte Adhesion Deficiency; Shwachman-Diamond Syndrome; Diamond-Blackfan Anemia; Dyskeratosis-congenita; Chediak-Higashi Syndrome; Severe Aplastic Anemia; Thalassemia Major; Hemophagocytic Lymphohistiocytosis; Sickle Cell Disease
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Recent advances in understanding clonal haematopoiesis in aplastic anaemia
Stanley, Natasha; Olson, Timothy S.; Babushok, Daria V.
2016-01-01
Summary Acquired aplastic anaemia (AA) is an immune-mediated bone marrow failure disorder inextricably linked to clonal haematopoiesis. The majority of AA patients have somatic mutations and/or structural chromosomal abnormalities detected as early as at diagnosis. In contrast to other conditions linked to clonal haematopoiesis, the clonal signature of AA reflects its immune pathophysiology. The most common alterations are clonal expansions of cells lacking glycophosphotidylinositol-anchored proteins, loss of human leucocyte antigen alleles, and mutations in BCOR/BCORL1, ASXL1 and DNMT3A. Here, we present the current knowledge of clonal haematopoiesis in AA as it relates to aging, inherited bone marrow failure, and the grey-zone overlap of AA and myelodysplastic syndrome (MDS). We conclude by discussing the significance of clonal haematopoiesis both for improved diagnosis of AA, as well as for a more precise, personalized approach to prognostication of outcomes and therapy choices. PMID:28107566
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Recent advances in understanding clonal haematopoiesis in aplastic anaemia.
Stanley, Natasha; Olson, Timothy S; Babushok, Daria V
2017-05-01
Acquired aplastic anaemia (AA) is an immune-mediated bone marrow failure disorder inextricably linked to clonal haematopoiesis. The majority of AA patients have somatic mutations and/or structural chromosomal abnormalities detected as early as at diagnosis. In contrast to other conditions linked to clonal haematopoiesis, the clonal signature of AA reflects its immune pathophysiology. The most common alterations are clonal expansions of cells lacking glycophosphotidylinositol-anchored proteins, loss of human leucocyte antigen alleles, and mutations in BCOR/BCORL1, ASXL1 and DNMT3A. Here, we present the current knowledge of clonal haematopoiesis in AA as it relates to aging, inherited bone marrow failure, and the grey-zone overlap of AA and myelodysplastic syndrome (MDS). We conclude by discussing the significance of clonal haematopoiesis both for improved diagnosis of AA, as well as for a more precise, personalized approach to prognostication of outcomes and therapy choices. © 2017 John Wiley & Sons Ltd.
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Protective environment for marrow transplant recipients. A prospective study
DOE Office of Scientific and Technical Information (OSTI.GOV)
Buckner, C.D.; Clift, R.A.; Sanders, J.E.
1978-12-01
Laminar air flow isolation and decontamination procedures were evaluated in a prospective randomized study in patients with aplastic anemia or acute leukemia undergoing marrow transplantation from HLA-matched siblings. Patients transplanted in the laminar air flow group had significantly less septicemia and major local infections than did patients in the control group. Nineteen of 46 laminar air flow patients and six of 44 control patients are alive at present. In patients with aplastic anemia the survival was 13 of 17 in the laminar air flow group compared with four of 17 in the control group. In patients with acute leukemia themore » survival was six of 29 in the laminar air flow group versus two of 27 in the control group. These differences were not statistically significant. Death in both the laminar air flow and control groups was predominantly due to interstitial pneumonitis or recurrent leukemia, which were unaffected by isolation and decontamination.« less
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Fluoxymesterone-induced gynaecomastia in a patient with childhood aplastic anaemia
Lo, Tom Edward Ngo; Andal, Zillien C; Lantion-Ang, Frances Lina
2015-01-01
Gynaecomastia is a benign condition characterised by enlargement of the male breast. Drug-induced gynaecomastia merits deep consideration as it may account for as many as 25% of all cases of gynaecomastia in adults. Although the mechanism is not fully clear, some mechanisms include oestrogen-like activities, stimulation of testicular production of oestrogens, inhibition of testosterone synthesis or blockade of androgen action. Anabolic steroids, in particular when used during the pubertal stage, may cause significant irreversible gynaecomastia. We report a case of 28-year-old Filipino man with persistent gynaecomastia from fluoxymesterone used for aplastic anaemia during his prepubertal stage. Hormonal work ups for gynaecomastia all turned out normal, thus isolating the drug as the cause. The patient was unable to undergo breast reconstruction surgery due to haematological contraindications, but eventually referred to psychiatry for counselling. This case will highlight the paradoxical effect of androgenic steroid used during childhood on male breast proliferation during puberty. PMID:25948845
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An apparent cluster of aplastic anemia in a small population of teenagers.
Linet, M S; Tielsch, J M; Markowitz, J A; Sensenbrenner, L L; McCaffrey, L D; Warm, S G; Vanderslice, S F; Morgan, W F; Bearden, J D; Szklo, M
1985-04-01
Four teenagers with severe aplastic anemia, initially diagnosed and evaluated over a seven-year period at The Johns Hopkins Bone Marrow Transplant Unit, Baltimore, were residents of the same small town in South Carolina. Estimated annual incidence for that age group in the town, based on the four cases, was 100 times the expected rate. All four of the teenagers had attended one of two junior high schools. An exploratory survey of all high-school students, comparing risk factors of those who had attended the "affected" junior high school with those who had attended the "unaffected" junior high school, showed no associations with exposure to glue, paint or varnishes, pesticides, history of hepatitis or infectious mononucleosis, or use of chloramphenicol or other suspected drugs. Weak associations were found between the affected junior high school and employment in the textile industry and in agriculture (specifically peach orchards).
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2018-06-13
Accelerated Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Acute Leukemia in Remission; Acute Lymphoblastic Leukemia; Acute Myeloid Leukemia; Acute Myeloid Leukemia With FLT3/ITD Mutation; Acute Myeloid Leukemia With Gene Mutations; Aplastic Anemia; B-Cell Non-Hodgkin Lymphoma; CD40 Ligand Deficiency; Chronic Granulomatous Disease; Chronic Leukemia in Remission; Chronic Lymphocytic Leukemia; Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Congenital Amegakaryocytic Thrombocytopenia; Congenital Neutropenia; Congenital Pure Red Cell Aplasia; Glanzmann Thrombasthenia; Immunodeficiency Syndrome; Myelodysplastic Syndrome; Myelofibrosis; Myeloproliferative Neoplasm; Paroxysmal Nocturnal Hemoglobinuria; Plasma Cell Myeloma; Polycythemia Vera; Recurrent Non-Hodgkin Lymphoma; Refractory Non-Hodgkin Lymphoma; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndrome; Severe Aplastic Anemia; Shwachman-Diamond Syndrome; Sickle Cell Disease; T-Cell Non-Hodgkin Lymphoma; Thalassemia; Waldenstrom Macroglobulinemia; Wiskott-Aldrich Syndrome
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ERIC Educational Resources Information Center
Eggen, Jerald D.; Mackenzie, R. Alec
1987-01-01
To avoid emergency-driven work environments, crises should be anticipated to the extent possible; steps should be taken to prevent them or limit their consequences. Crises have both internal (personal) and external causes based on poor performance, unreasonable demands, or shifting priorities. Administrators need to cushion deadlines, clarify…
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The impact of economic crises on social inequalities in health: what do we know so far?
Bacigalupe, Amaia; Escolar-Pujolar, Antonio
2014-07-25
Since 2008, Western countries are going through a deep economic crisis whose health impacts seem to be fundamentally counter-cyclical: when economic conditions worsen, so does health, and mortality tends to rise. While a growing number of studies have presented evidence on the effect of crises on the average population health, a largely neglected aspect of research is the impact of crises and the related political responses on social inequalities in health, even if the negative consequences of the crises are primarily borne by the most disadvantaged populations. This commentary will reflect on the results of the studies that have analyzed the effect of economic crises on social inequalities in health up to 2013. With some exceptions, the studies show an increase in health inequalities during crises, especially during the Southeast Asian and Japanese crises and the Soviet Union crisis, although it is not always evident for both sexes or all health or socioeconomic variables. In the Nordic countries during the nineties, a clear worsening of health equity did not occur. Results about the impacts of the current economic recession on health equity are still inconsistent. Some of the factors that could explain this variability in results are the role of welfare state policies, the diversity of time periods used in the analyses, the heterogeneity of socioeconomic and health variables considered, the changes in the socioeconomic profile of the groups under comparison in times of crises, and the type of measures used to analyze the magnitude of social inequalities in health. Social epidemiology should further collaborate with other disciplines to help produce more accurate and useful evidence about the relationship between crises and health equity.
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Bradley, Melville D
2011-03-01
2,4,6-Trinitrotoluene (TNT) is an explosive used in munitions production that is known to cause both aplastic and hemolytic anemia in exposed workers. Anemia in a TNT worker is considered a sentinel health event (occupational) (SHE(O)) in the United States (US). Deaths have been reported secondary to aplastic anemia. Studies have shown that TNT systemic absorption is significant by both the respiratory and dermal routes. No studies encountered looked at hemoglobin change or anemia cases in respiratory protected workers. It is hypothesized that respiratory protection is insufficient to protect TNT workers from the risk of anemia development and hemoglobin concentration drop. A records review of eight groups of respiratory protected TNT workers' pre-exposure hemoglobin levels were compared with their during-exposure hemoglobin levels for statistically significant (alpha level 0.05) hemoglobin level changes, and anemia cases were recorded. A curve estimation analysis was performed between mean TNT air concentrations and mean hemoglobin change values. Statistically significant hemoglobin level drops and anemia cases were apparent at TNT air concentrations about the REL and PEL in respiratory protected workers. There were no anemia cases or statistically significant hemoglobin level drops at concentrations about the TLV, however. A statistically significant inverse non-linear regression model was found to be the best fit for regressing hemoglobin change on TNT air concentration. Respiratory protection may be inadequate to prevent workers who are at risk for TNT skin absorption from developing anemia. This study contributes evidence that the TLV should be considered for adoption as the new PEL.
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Wu, Dijiong; Wen, Xiaowen; Liu, Wenbin; Hu, Huijin; Ye, Baodong; Zhou, Yuhong
2018-01-01
Iron overload is commonly observed during the course of aplastic anemia (AA), which is believed to aggravate hematopoiesis, cause multiple organ dysfunction, lead to disease progression, and impair quality of life. Deferasirox (DFX) and deferoxamine (DFO) are among the most common iron chelation agents available in the clinical setting. The aim of this study was to investigate if the combination therapy with DFX and DFO is superior in hematopoietic recovery and iron chelation. Briefly, we developed a composite mouse model with AA and iron overload that was consequently treated with DFX, DFO, or with a combination of both agents. The changes in peripheral hemogram, marrow apoptosis, and its related protein expressions were compared during the process of iron chelation, while the iron depositions in liver and bone marrow and its regulator were also detected. The obtained results showed that compared to DFX, DFO has a better effect in protecting the bone marrow from apoptosis-induced failure. The combination of DFO and DFX accelerated the chelation of iron, while their efficiency on further hemogram improvement appeared limited. To sum up, our data suggest that single treatment with DFO may be a better choice for improving the hematopoiesis during the gradual chelation treatment irrespective of the convenience of oral DFX, while the combination treatment should be considered for urgent reduction of the iron burden.
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Chen, Chi-Ching; Chen, Chin-Shan; Wang, Wei-Yao; Ma, Jui-Shan; Shu, Hwei-Fan; Fan, Frank S
2015-03-12
Parvovirus B19 virus commonly causes subclinical infection, but it can prove fatal to the fetus during pregnancy and cause severe anemia in an adult with hemolytic diseases. We present the case of a woman with autoimmune hemolytic anemia who was diagnosed with parvovirus B19-induced transient aplastic crisis during her second trimester of pregnancy and faced the high risk of both fetal and maternal complications related to this specific viral infection. To the best of our knowledge, the experience of successful intravenous immunoglobulin treatment for B19 virus infection during pregnancy, as in our case, is limited. A 28-year-old and 20-week pregnant Chinese woman with genetically confirmed alpha-thalassemia trait was diagnosed with cold antibody autoimmune hemolytic anemia and suffered from transient aplastic crisis caused by B19 virus infection. She received intravenous immunoglobulin treatment to reduce the risk of hydrops fetalis. Her peripheral blood reticulocyte percentage recovered, but anemia persisted, so she underwent several courses of high dose intravenous dexamethasone for controlling her underlying hemolytic problem. Finally, her hemoglobin levels remained stable with no need of erythrocyte transfusion, and a healthy baby boy was naturally delivered. Parvovirus B19 virus infection should be considered when a sudden exacerbation of anemia occurs in a patient with hemolytic disease, and the possible fetal complications caused by maternal B19 virus infection during pregnancy should not be ignored. Close monitoring and adequate management can keep both mother and fetus safe.
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Managing Mental Health Crises of Foreign College Students.
ERIC Educational Resources Information Center
Oropeza, Barbara A. Clark; And Others
1991-01-01
Notes that student services professionals manage a number of mental health crises as part of their job responsibilities. Examines some issues that arise from assisting foreign college students experiencing such crises, with special focus on psychiatric committal, withdrawal from school, and return to the home country. (Author)
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Code of Federal Regulations, 2014 CFR
2014-01-01
...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...
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Code of Federal Regulations, 2010 CFR
2010-01-01
...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...
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Code of Federal Regulations, 2011 CFR
2011-01-01
...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...
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Code of Federal Regulations, 2013 CFR
2013-01-01
...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...
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Code of Federal Regulations, 2012 CFR
2012-01-01
...] (H) Severe cardiomyopathy—Class IV. (I) Aplastic anemia. (J) Uncontrolled hypertension with...) Severe hepatic failure. (N) Severe Hypoxic brain damage. (O) Severe portal hypertension with esophageal...
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The relationship of aplastic anemia and PNH.
Young, Neal S; Maciejewski, Jaroslaw P; Sloand, Elaine; Chen, Guiben; Zeng, Weihua; Risitano, Antonio; Miyazato, Akira
2002-08-01
Bone marrow failure has been regarded as one of the triad of clinical manifestations of paroxysmal noctumal hemoglobinuria (PNH), and PNH in turn has been described as a late clonal disease evolving in patients recovering from aplastic anemia. Better understanding of the pathophysiology of both diseases and improved tests for cell surface glycosylphosphatidylinositol (GPI)-linked proteins has radically altered this view. Flow cytometry of granulocytes shows evidence of an expanded PNH clone in a large proportion of marrow failure patients at the time of presentation: in our large NIH series, about 1/3 of over 200 aplastic anemia cases and almost 20% of more than 100 myelodysplasia cases. Clonal PNH expansion (rather than bone marrow failure) is strongly linked to the histocompatability antigen HLA.-DR2 in all clinical varieties of the disease, suggesting an immune component to its pathophysiology. An extrinsic mechanism of clonal expansion is also more consistent with knock-out mouse models and culture experiments with primary cells and cell lines, which have failed to demonstrate an intrinsic proliferative advantage for PNH cells. DNA chip analysis of multiple paired normal and PIG-A mutant cell lines and lymphoblastoid cells do not show any consistent differences in levels of gene expression. In aplastic anemia/PNH there is surprisingly limited utilization of the V-beta chain of the T cell receptor, and patients' dominant T cell clones, which are functionally inhibitory of autologous hematopoiesis, use identical CDR3 regions for antigen binding. Phenotypically normal cells from PNH patients proliferate more poorly in culture than do the same patient's PNH cells, and the normal cells are damaged as a result of apoptosis and overexpress Fas. Differences in protein degradation might play a dual role in pathophysiology, as GPI-linked proteins lacking an anchor would be predicted to be processed by the proteasome machinery and displayed in a class I H.A. context, in contrast to the normal pathway of cell surface membrane recycling, lysosomal degradation, and presentation by class II HLA. The strong relationship between a chronic, organ-specific immune destructive process and the expansion of a single mutant stem cell clone remains frustratingly enigmatic but likely to be the result of interesting biologic processes, with mechanisms that potentially can be extended to the role of inflammation in producing premalignant syndromes.
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Duda, Alfred M
2003-12-29
Interlinked crises of land degradation, food security, ecosystem decline, water quality and water flow depletion stand in the way of poverty reduction and sustainable development. These crises are made worse by increased fluctuations in climatic regimes. Single-purpose international conventions address these crises in a piecemeal, sectoral fashion and may not meet their objectives without greater attention to policy, legal, and institutional reforms related to: (i) balancing competing uses of land and water resources within hydrologic units; (ii) adopting integrated approaches to management; and (iii) establishing effective governance institutions for adaptive management within transboundary basins. This paper describes this global challenge and argues that peace, stability and security are all at stake when integrated approaches are not used. The paper presents encouraging results from a decade of transboundary water projects supported by the Global Environment Facility in developing countries that test practical applications of processes for facilitating reforms related to land and water that are underpinned by science-based approaches. Case studies of using these participative processes are described that collectively assist in the transition to integrated management. A new imperative for incorporating interlinkages among food, water, and environment security at the basin level is identified.
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Duda, Alfred M
2003-01-01
Interlinked crises of land degradation, food security, ecosystem decline, water quality and water flow depletion stand in the way of poverty reduction and sustainable development. These crises are made worse by increased fluctuations in climatic regimes. Single-purpose international conventions address these crises in a piecemeal, sectoral fashion and may not meet their objectives without greater attention to policy, legal, and institutional reforms related to: (i) balancing competing uses of land and water resources within hydrologic units; (ii) adopting integrated approaches to management; and (iii) establishing effective governance institutions for adaptive management within transboundary basins. This paper describes this global challenge and argues that peace, stability and security are all at stake when integrated approaches are not used. The paper presents encouraging results from a decade of transboundary water projects supported by the Global Environment Facility in developing countries that test practical applications of processes for facilitating reforms related to land and water that are underpinned by science-based approaches. Case studies of using these participative processes are described that collectively assist in the transition to integrated management. A new imperative for incorporating interlinkages among food, water, and environment security at the basin level is identified. PMID:14728798
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Developing Community College Faculty as Leaders.
ERIC Educational Resources Information Center
Cooper, Joanne E.; Pagotto, Louise
2003-01-01
Discusses the national crises of community college leadership in America. Describes the role of faculty members in community college leadership. Describes motivational factors involved in college leadership, some leadership challenges, leadership development initiatives, and some opportunities for faculty leadership development. Also describes two…
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Kovoor-Misra, S.
Technical organizations are not only vulnerable to crises, but have the potential to create catastrophic crises. This has been devastatingly illustrated by recent crises, such as the gas leak in Bhopal, India, the Exxon Valdez oil spill, and the Challenger explosion. Thus, crises preparation for these organizations. This paper describes a Multidimensional Approach to crisis preparation for technical organizations. As part of this approach, come critical factors for crisis preparation are also described. The Multidimensional Approach is derived from the literature and a study of the crisis preparation of nine technical organizations. 28 refs., 3 figs., 4 tab.
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Impact of the topology of global macroeconomic network on the spreading of economic crises.
Lee, Kyu-Min; Yang, Jae-Suk; Kim, Gunn; Lee, Jaesung; Goh, Kwang-Il; Kim, In-mook
2011-03-31
Throughout economic history, the global economy has experienced recurring crises. The persistent recurrence of such economic crises calls for an understanding of their generic features rather than treating them as singular events. The global economic system is a highly complex system and can best be viewed in terms of a network of interacting macroeconomic agents. In this regard, from the perspective of collective network dynamics, here we explore how the topology of the global macroeconomic network affects the patterns of spreading of economic crises. Using a simple toy model of crisis spreading, we demonstrate that an individual country's role in crisis spreading is not only dependent on its gross macroeconomic capacities, but also on its local and global connectivity profile in the context of the world economic network. We find that on one hand clustering of weak links at the regional scale can significantly aggravate the spread of crises, but on the other hand the current network structure at the global scale harbors higher tolerance of extreme crises compared to more "globalized" random networks. These results suggest that there can be a potential hidden cost in the ongoing globalization movement towards establishing less-constrained, trans-regional economic links between countries, by increasing vulnerability of the global economic system to extreme crises.
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Impact of the Topology of Global Macroeconomic Network on the Spreading of Economic Crises
Lee, Kyu-Min; Yang, Jae-Suk; Kim, Gunn; Lee, Jaesung; Goh, Kwang-Il; Kim, In-mook
2011-01-01
Throughout economic history, the global economy has experienced recurring crises. The persistent recurrence of such economic crises calls for an understanding of their generic features rather than treating them as singular events. The global economic system is a highly complex system and can best be viewed in terms of a network of interacting macroeconomic agents. In this regard, from the perspective of collective network dynamics, here we explore how the topology of the global macroeconomic network affects the patterns of spreading of economic crises. Using a simple toy model of crisis spreading, we demonstrate that an individual country's role in crisis spreading is not only dependent on its gross macroeconomic capacities, but also on its local and global connectivity profile in the context of the world economic network. We find that on one hand clustering of weak links at the regional scale can significantly aggravate the spread of crises, but on the other hand the current network structure at the global scale harbors higher tolerance of extreme crises compared to more “globalized” random networks. These results suggest that there can be a potential hidden cost in the ongoing globalization movement towards establishing less-constrained, trans-regional economic links between countries, by increasing vulnerability of the global economic system to extreme crises. PMID:21483794
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Mobile Response Team Saves Lives in Volcano Crises
Ewert, John W.; Miller, C. Dan; Hendley, James W.; Stauffer, Peter H.
1997-01-01
The world's only volcano crisis response team, organized and operated by the USGS, can be quickly mobilized to assess and monitor hazards at volcanoes threatening to erupt. Since 1986, the team has responded to more than a dozen volcano crises as part of the Volcano Disaster Assistance Program (VDAP), a cooperative effort with the Office of Foreign Disaster Assistance of the U.S. Agency for International Development. The work of USGS scientists with VDAP has helped save countless lives, and the valuable lessons learned are being used to reduce risks from volcano hazards in the United States.
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Use of verbal autopsy and social autopsy in humanitarian crises.
Thomas, Lisa-Marie; D'Ambruoso, Lucia; Balabanova, Dina
2018-01-01
Two billion people live in countries affected by conflict, violence and fragility. These are exceptional situations in which mortality shifts dramatically and in which civil registration and vital statistics systems are often weakened or cease to function. Verbal autopsy and social autopsy (VA and SA) are methods used to assign causes of death and understand the contexts in which these occur, in settings where information is otherwise unavailable. This review sought to explore the use of VA and SA in humanitarian crises, with a focus on how these approaches are used to inform policy and programme responses. A rapid scoping review was conducted on the use of VA and SA in humanitarian crises in low and middle-income countries since 1991. Drawing on a maximum variation approach, two settings of application ('application contexts') were selected and investigated via nine semi-structured expert interviews. VA can determine causes of death in crisis-affected populations where no other registration system is in place. Combined with SA and active community involvement, these methods can deliver a holistic view of obstacles to seeking and receiving essential healthcare, yielding context-specific information to inform appropriate responses. The contexts in which VA and SA are used require adaptations to standard tools, and new mobile developments in VA raise specific ethical considerations. Furthermore, collecting and sythesising data in a timely, continuous manner, and ensuring coordination and communication between agencies, is important to realise the potential of these approaches. VA and SA are valuable research methods to foster evidence-informed responses for populations affected by humanitarian crises. When coordinated and communicated effectively, data generated through these methods can help to identify levels, causes and circumstances of deaths among vulnerable groups, and can enable planning and allocating resources effectively, potentially improving health system resilience to future crises.
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Introduction: evidence-based action in humanitarian crises.
Dijkzeul, Dennis; Hilhorst, Dorothea; Walker, Peter
2013-07-01
This introductory paper sets the stage for this special issue of Disasters on evidence-based action in humanitarian crises. It reviews definition(s) of evidence and it examines the different disciplinary and methodological approaches to collecting and analysing evidence. In humanitarian action, the need for evidence-based approaches sometimes is viewed in tension with a principled approach, often unnecessarily. Choosing appropriate research methods depends on the objectives of the researcher, in particular whether the research focuses on the intervention and/or the context and the length and complexity of the causal chains involved. The paper concludes by defining some trends in evidence-based approaches in crises: the move away from inputs and outputs of humanitarian action towards outcomes and impacts; the shift towards a higher degree of partnerships in research, and the participation of users and target groups; and the acceptance of a broad array of approaches to establish evidence. © 2013 The Author(s). Journal compilation © Overseas Development Institute, 2013.
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Federal Register 2010, 2011, 2012, 2013, 2014
2012-07-13
... most serious being bone marrow depression (anemia, thrombocytopenia, and granulocytopenia temporally... succinate injection and chloramphenicol capsules states that serious hypoplastic anemia, thrombocytopenia... anemia associated with administration of the drug and aplastic anemia attributed to chloramphenicol that...
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2016-01-28
Acute Myelogenous Leukemia; Acute Lymphocytic Leukemia; Chronic Myelogenous Leukemia; Chronic Lymphocytic Leukemia; Myelodysplasia; Non-Hodgkin's Lymphoma; Hodgkin's Disease; Multiple Myeloma; Myelofibrosis; Anemia, Aplastic; Hemoglobinuria, Paroxysmal
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Snarski, Emilian; Milczarczyk, Alicja; Franek, Edward; Jedrzejczak, Wieslaw
2010-01-01
Immunoablation with autologous hematopoietic cell transplantation has shown some effectiveness in the treatment of autoimmune diseases as diverse as aplastic anemia, systemic lupus erythematosus, multiple sclerosis and Crohn's disease. It has been recently shown that this treatment might prevent or delay development of diabetes type 1. The majority of more than 30 patients with early diabetes type 1 who underwent immunoablation and hematopoietic cell transplantation in various centers in the world achieved durable remission of diabetes and independence of exogenous insulin. This review summarizes advantages and risks of this treatment of early diabetes type 1.
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ERIC Educational Resources Information Center
Kisch, Marian
2012-01-01
Natural disasters, as well as crises of the man-made variety, call on leaders of school districts to manage scenarios impossible to predict and for which no amount of training can adequately prepare. One thing all major crises hold in common is their far-reaching effects, which can run the gamut from personal safety and mental well-being to the…
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Helping Students Cope with Fears and Crises.
ERIC Educational Resources Information Center
Walz, Garry R., Ed.; Bleuer, Jeanne C., Ed.
This document consists of two modules extracted from a six-module larger work. Module 1 presents six articles on the topic of "helping students to cope with fears and crises." Module 2 contains 17 articles on "programs and practices for helping students cope with fears and crises." Article titles and authors are as follows: (1)…
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Rural Development: Goals, Dynamics, Crises and Recommendations.
ERIC Educational Resources Information Center
Jehlik, Paul J.
The continuing transition of rural development from its old to present day form is discussed, treating varied perceptions of its goals and the continuing resolution of approaches through legislation and appropriations. Some of the goals are community development, human resources development, natural resources preservation, and a more equitable…
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THE IMPACT OF CRISIS UPON THE BEHAVIOR OF NATIONS IN THE 1960's.
Phillips, W R; Lorimor, T
1974-10-01
This study examines international crises in the 1960's, specifically those crises that occurred between June, 1962 and July, 1968. The conflictual aspects of interacting dyads involved in crises during that period were examined t o discern the conflictual dimensions of national behavior in crises. In addition, conflict behavior was examined for those periods immediately prior to and immediately following crisis situations. This resulted in dimensions of conflict behavior for pre-crisis, during-crisis, and post-crisis periods. The periods were contrasted with one another to gain a better conception of the behavior exhibited in international crises. It was found that the dimensions of behavior change in predictable ways in the shift from one period to another. The behavior of individual dyads does shift in ways which are not predictable by simple linear technique.
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Cylus, Jonathan; Mladovsky, Philipa; McKee, Martin
2012-01-01
Objective To identify whether, by what means, and the extent to which historically, government health care expenditure growth in Europe has changed following economic crises. Data Sources Organization for Economic Cooperation and Development Health Data 2011. Study Design Cross-country fixed effects multiple regression analysis is used to determine whether statutory health care expenditure growth in the year after economic crises differs from that which would otherwise be predicted by general economic trends. Better understanding of the mechanisms involved is achieved by distinguishing between policy responses which lead to cost-shifting and all others. Findings In the year after an economic downturn, public health care expenditure grows more slowly than would have been expected given the longer term economic climate. Cost-shifting and other policy responses are both associated with these slowdowns. However, while changes in tax-derived expenditure are associated with both cost-shifting and other policy responses following a crisis, changes in expenditure derived from social insurance have been associated only with changes in cost-shifting. Conclusions Disproportionate cuts to the health sector, as well as reliance on cost-shifting to slow growth in health care expenditure, serve as a warning in terms of potentially negative effects on equity, efficiency, and quality of health services and, potentially, health outcomes following economic crises. PMID:22670771
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Cylus, Jonathan; Mladovsky, Philipa; McKee, Martin
2012-12-01
To identify whether, by what means, and the extent to which historically, government health care expenditure growth in Europe has changed following economic crises. Organization for Economic Cooperation and Development Health Data 2011. Cross-country fixed effects multiple regression analysis is used to determine whether statutory health care expenditure growth in the year after economic crises differs from that which would otherwise be predicted by general economic trends. Better understanding of the mechanisms involved is achieved by distinguishing between policy responses which lead to cost-shifting and all others. In the year after an economic downturn, public health care expenditure grows more slowly than would have been expected given the longer term economic climate. Cost-shifting and other policy responses are both associated with these slowdowns. However, while changes in tax-derived expenditure are associated with both cost-shifting and other policy responses following a crisis, changes in expenditure derived from social insurance have been associated only with changes in cost-shifting. Disproportionate cuts to the health sector, as well as reliance on cost-shifting to slow growth in health care expenditure, serve as a warning in terms of potentially negative effects on equity, efficiency, and quality of health services and, potentially, health outcomes following economic crises. © Health Research and Educational Trust.
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The end of the era of generosity? Global health amid economic crisis
Schneider, Kammerle; Garrett, Laurie
2009-01-01
In the past decade donor commitments to health have increased by 200 percent. Correspondingly, there has been a swell of new players in the global health landscape. The unprecedented, global response to a single disease, HIV/AIDS, has been responsible for a substantial portion of this boon. Numerous health success have followed this windfall of funding and attention, yet the food, fuel, and economic crises of 2008 have shown the vulnerabilities of health and development initiatives focused on short term wins and reliant on a constant flow of foreign funding. For too long, the international community has responded to global health and development challenges with emergency solutions that often reflect the donor's priorities, values, and political leanings, rather than funding durable health systems that can withstand crises. Progress towards achieving the Millennium Development Goals has stalled in many countries. Disease specific initiatives have weakened health systems and limited efforts to improve maternal and child health. As we enter this era of scarce resources, there is a need to return to the foundations of the Alma Ata Declaration signed thirty years ago with the goal of providing universal access to primary healthcare. The global health community must now objectively evaluate how we can most effectively respond to the crises of 2008 and take advantage of this moment of extraordinary attention for global health and translate it into long term, sustainable health improvements for all. PMID:19134211
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2017-06-01
Vitamin D Deficiency; Stem Cell Transplant Complications; Pediatric Cancer; Blood Disorder; Pediatric Acute Myeloid Leukemia; Pediatric Acute Lymphoid Leukemia; Myelodysplastic Syndromes; Sickle Cell Anemia in Children; Aplastic Anemia; Thalassemia in Children
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Causes, Consequences and Control of Students' Crises in Public and Private Universities in Nigeria
ERIC Educational Resources Information Center
Adeyemi, T. O.
2009-01-01
This paper investigated the causes, consequences and control of students' crises in public and private universities in Nigeria. Students' crises involve making protest by students' in pressing their demand on various issues with university authorities. In this regard, the study population comprised all the 81 universities in the country from which…
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Shukor, Ali R; Klazinga, Niek S; Kringos, Dionne S
2017-08-23
This study presents a descriptive synthesis of Kurdistan Region of Iraq's (KRI) primary care system, which is undergoing comprehensive primary care reforms within the context of a cross-cutting structural economic adjustment program and protracted security, humanitarian, economic and political crises. The descriptive analysis used a framework operationalizing Starfield's classic primary care model for health services research. A scoping review was performed using relevant sources, and expert consultations were conducted for completing and validating data. The descriptive analysis presents a complex narrative of a primary care system undergoing classical developmental processes of transitioning middle-income countries. The system is simultaneously under tremendous pressure to adapt to the continuously changing, complex and resource-intensive needs of sub-populations exhibiting varying morbidity patterns, within the context of protracted security, humanitarian, economic, and political crises. Despite exhibiting significant resilience in the face of the ongoing crises, the continued influx of IDPs and Syrian refugees, coupled with extremely limited resources and weak governance at policy, organizational and clinical levels threaten the sustainability of KRI's public primary care system. Diverse trajectories to the strengthening and development of primary care are underway by local and international actors, notably the World Bank, RAND Corporation, UN organizations and USAID, focusing on varying imperatives related to the protracted humanitarian and economic crises. The convergence, interaction and outcomes of the diverse initiatives and policy approaches in relation to the development of KRI's primary care system are complex and highly uncertain. A common vision of primary care is required to align resources, initiatives and policies, and to enable synergy between all local and international actors involved in the developmental and humanitarian response. Further research that integrates the knowledge synthesized in this article, and enables actors in KRI to learn from their own experiences and efforts, along with those of other jurisdictions, would be invaluable towards the ongoing development of primary care.
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Peffault de Latour, Régis; Tabrizi, Reza; Marcais, Ambroise; Leblanc, Thierry; Lamy, Thierry; Mohty, Mohamad; Tavitian, Suzanne; Jubert, Charlotte; Pasquet, Marlène; Galambrun, Claire; Nguyen, Stéphanie; Cahn, Jean Yves; Braun, Thorsten; Deconinck, Eric; Bay, Jacques Olivier; Sicre de Fontbrune, Flore; Barraco, Fiorenza; Socié, Gérard
2018-05-01
Antithymocyte globulins (ATG) plus cyclosporine (CSA) is the gold standard immunosuppressive treatment (IST) for patients with aplastic anemia. A prospective randomized trial showed in 2011 that hATG was superior to rabbit ATG for first-line treatment of severe AA. The French Health Agency (ANSM) permitted a patient-named authorization for temporary use (ATU) program of hATG (ATGAM, Pfizer) in patients with AA in 2011 since commercial access to hATG is not approved. We took advantage of this program to analyze the outcomes of 465 patients who received antithymocyte globulins (ATGAM) plus CSA as first line treatment (n = 379; 81.5%), or for refractory (n = 26) or relapsed disease (n = 33), from September 2011 to March 2017. In the entire cohort one year, 72% of the patients had partial and 13% had complete response, with worse response for patients with severe AA and a longer interval between diagnosis and IST (more than 6 months). Severe adverse events were mainly linked to infections (24%), hemorrhages (6%), and elevated liver function tests (5%). Overall at 12 months, 9.7% of patients required second line IST and 15.6% received transplantation. Fifty-five patients died during the study mainly because of infections (53%). Factors predicting independently worse survival were age over 40 years, neutrophils less than 0.5 × 10 9 /L, male gender and longer delay between diagnosis and hATG (>6 months period). This study does illustrate the results of ATGAM with CSA in a true-life perspective and confirms ATGAM as standard of care IST to treat patients with AA not eligible for HSCT. © 2018 Wiley Periodicals, Inc.
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Kobayashi, Yujin; Hatta, Yoshihiro; Ishiwatari, Yusaku; Kanno, Hitoshi; Takei, Masami
2014-03-11
Although there are several case reports of human parvovirus B19 infection in patients with hereditary spherocytosis, no systematic reviews of adult patients with hereditary spherocytosis with human parvovirus B19 infection have been published as clinical case reports. In this study, we report a case of aplastic crisis due to human parvovirus B19 infection in an adult patient with hereditary spherocytosis. A 33-year-old woman with hereditary spherocytosis and gallstones was admitted because of rapid progress in marked anemia and fever. Although empiric antibiotic therapy was prescribed, her clinical symptoms and liver function test worsened. Because the anti-human parvovirus B19 antibody and deoxyribonucleic acid levels assessed by polymerase chain reaction were positive, the patient was diagnosed with aplastic crisis due to the human parvovirus B19 infection. We collected and reviewed several case reports of patients with hereditary spherocytosis aged > 18 years with human parvovirus B19 infection between 1984 and 2010. A total of 19 reports with 22 cases [median age, 28 years (range, 18-43 range); male: female ratio, 6:16], including the present case were identified. The male-to-female ratio of 6:16 implied that younger females were predominantly affected. Although fever and abdominal symptoms were common initial symptoms, liver dysfunction or skin eruptions were less commonly documented. Anti-human parvovirus B19 antibody or deoxyribonucleic acid levels assessed by polymerase chain reaction was commonly used to diagnose human parvovirus B19 infection and may be useful to distinguish human parvovirus B19 infection from other abdominal infection in patients with hereditary spherocytosis.
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Zhang, Xia; Li, Zhangzhi; Geng, Wei; Song, Bin; Wan, Chucheng
2018-07-01
To investigate the efficacy and safety of umbilical cord blood (UCB) infusion (UCBI) plus immunosuppressive therapy (IST) treatment in comparison to IST treatment, as well as predictive factors for clinical responses, in severe aplastic anemia (SAA) patients. Totally, 93 patients with SAA were enrolled in this cohort study. In the IST group, rabbit antithymocyte globulin (r-ATG) combined with cyclosporine A (CsA) was administered, while in the IST+UBCI group, r-ATG, CsA, and UCB were used. After 6 months of treatment, UCBI+IST achieved a higher complete response (CR) rate (p=0.002) and an elevated overall response rate (ORR) (p=0.004), compared to IST. Regarding hematopoietic recovery at month 6, platelet responses in the UCBI+IST group were better than those in the IST group (p=0.002), and UCBI+IST treatment facilitated increasing trends in absolute neutrophil count (ANC) response (p=0.056). Kaplan-Meier curves illuminated UCBI+IST achieved faster ANC response (p<0.001) and platelet response (p<0.001), compared with IST therapy. There was no difference in overall survival (OS) between the two groups (p=0.620). Furthermore, logistic regression analysis demonstrated that UCBI+IST was an independent predicting factor for both CR (p=0.001) and ORR (p<0.001), compared to IST; meanwhile, very severe aplastic anemia (VSAA) and ANC could predict clinical responses as well. However, Cox proportional hazard regression indicated that VSAA (p=0.003), but not UCBI+IST, affected OS. Safety profiles showed that UCBI+IST therapy did not elevate adverse events, compared with IST treatment. UCBI+IST achieved better clinical responses and hematopoietic recovery than IST, and was well tolerated in SAA patients. © Copyright: Yonsei University College of Medicine 2018.
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Pancytopenia in two national ethnic groups of Baluchistan.
Tareen, Saleh Muhammad; Bajwa, Masroor Ahmad; Tariq, Mohammad Masood; Babar, Shakeel; Tareen, Abdul Malik
2011-01-01
Pancytopenia is a recognisable haematological problem with common categorises diagnosis but best possible diagnostic approach is still to be defined. The aim of this study was to determine the frequent causes and clinical presentation of pancytopenia in relation to age and sex in different ethnic groups of two national (Pakistani and Afghan) living in Baluchistan province. This is a cross sectional descriptive type of study was carried out on patients (n = 180) for two years (July 2009-June 2011). Cases were successively registered in 2 provincial government hospital and 2 private clinics in Quetta with diagnosis of pancytopenia. The most frequent causes of pancytopenia in both national ethnic groups were malaria (29.44%) fallowed by tuberculosis (17.22%), leukaemia (16.67%), aplastic anaemia (13.33%), hepatitis (12.22%), other diseases (7.22%) and iron deficiency anaemia (3.89%). Overall, 63.89% male subjects were observed pancytopenia as compared to 36.11% female patients. The results also showed that patients with age > 41 years were highly (50%) affected by pancytopenia followed by 21-40 years (30.66%) and < 20 years (19.44%). Moreover, leukaemia cases were observed significantly higher in Afghan subjects (> 41 years). The most common symptom of patients was (71.11%) fever followed by Pallor (42.22%), fatigue (38.33%), weight loss (26.11%) and dizziness (25.56%). Leukaemia and aplastic anaemia were found to be the most serious causes of pancytopenia in Afghan ethnic group. Malaria, tuberculosis, aplastic anaemia, and leukaemia were the leading causes of pancytopenia in Pakistani and Afghan ethnic groups. Severe pancytopenia has significant relation with the clinical conclusion and can be used as a prognostic marker.
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Multiple crises and global health: New and necessary frontiers of health politics
Schrecker, Ted
2012-01-01
The world economy is entering an era of multiple crises, involving finance, food security and global environmental change. This article assesses the implications for global public health, describes the contours of post-2007 crises in food security and finance, and then briefly indicates the probable health impacts. There follows a discussion of the crisis of climate change, one that will unfold over a longer time frame but with manifestations that may already be upon us. The article then discusses the political economy of responses to these crises, noting the formidable obstacles that exist to equitable resolution. The article concludes by noting the threat that such crises present to recent progress in global health, arguing that global health researchers and practitioners must become more familiar with the relevant social processes, and that proposed solutions that neglect the continuing importance of the nation-state are misdirected. PMID:22657093
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Multiple crises and global health: new and necessary frontiers of health politics.
Schrecker, Ted
2012-01-01
The world economy is entering an era of multiple crises, involving finance, food security and global environmental change. This article assesses the implications for global public health, describes the contours of post-2007 crises in food security and finance, and then briefly indicates the probable health impacts. There follows a discussion of the crisis of climate change, one that will unfold over a longer time frame but with manifestations that may already be upon us. The article then discusses the political economy of responses to these crises, noting the formidable obstacles that exist to equitable resolution. The article concludes by noting the threat that such crises present to recent progress in global health, arguing that global health researchers and practitioners must become more familiar with the relevant social processes, and that proposed solutions that neglect the continuing importance of the nation-state are misdirected.
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Why is it necessary to examine retina when the patient suffers from aplastic anemia?
Tomcikova, D; Gerinec, A; Busanyova, B; Gresikova, M; Biskup, S; Hortnagel, K
2018-01-01
To inform about a case of Revesz syndrome (RS) with initial ophthalmological symptomatology of severe proliferative vitreoretinopathy of the left eye (LE). After the aplastic anemia had developed, RS was established. The exudative retinopathy was successfully treated with photocoagulation on the right eye (RE). RS is characterized by fatal bone marrow failure, exudative retinopathy, neuroradiographic abnormalities, neurodevelopmental delay and skin abnormalities. Non-treated exudative retinopathy leads to blindness. We report ophthalmological findings as follows: fundus photography and fluorescein angiography (FA) acquired by examinations under general anesthesia in patient with RS. Results of genetic tests helped to establish the diagnosis. Two‑year old Caucasian male was examined due to total retinal detachment on LE and signs of chorioretinal scarring on RE. In preoperative screening, thrombocytopenia was detected; later, severe pancytopenia developed. Considering the hematological findings and clinical appearance, we suspected RS, which was confirmed by genetic tests. We found a pathogenic mutation in gene TINF2 (variant c.865C>T;p.Pro289Ser) in a mosaic state with autosomal dominant mode of inheritance. This mutation has not been described in RS yet. Blind LE was enucleated because of dolorous neovascular glaucoma. FA of RE shows excessive areas of capillary nonperfusion with vascular abnormalities and exudation. After the photocoagulation, the visual acuity (VA) on RE remains 0.9 at the age of 7 years. RS is an extremely rare condition. The initial symptomatology could be ophthalmological or hematological. The positive finding of TINF2 gene mutation helped in establishing the correct diagnosis. The ischemic retinopathy was successfully treated by photocoagulation (Fig. 6, Ref. 6). Text in PDF www.elis.sk.
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Groth, Martha; Singer, Susanne; Niedeggen, Cathrin; Petermann-Meyer, Andrea; Röth, Alexander; Schrezenmeier, Hubert; Höchsmann, Britta; Brümmendorf, Tim H; Panse, Jens
2017-02-01
Acquired aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) are interrelated ultra-rare diseases. Quality of life (QoL) evaluation tools used in studies for AA and PNH are unspecific and designed for cancer patients (e.g., the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire, EORTC QLQ-C30). Given the complexity of AA and PNH, variation in symptoms and treatments, younger age of many patients, and the fact that AA and PNH are not classified as malignant diseases, it is likely that cancer-specific questionnaires are inappropriate. We generate an AA/PNH-specific QoL questionnaire (QLQ-AA/PNH), performed according to EORTC guidelines. QoL issues were obtained from the literature and interviews with patients and physicians (phase I), then ranked by patients and physicians. In phase II, items were created. Patients in more than 25 German and Swiss cities were interviewed face to face. In phase I, interviews of 19 patients and 8 physicians specialized in AA/PNH treatment resulted in 649 QoL issues; these were condensed to 175 and graded according to their importance by 30 patients and 14 physicians (phase II). Five physicians took part in phases I and II. Altogether, 97 issues were rated important. Twelve EORTC QLQ-C30 items were not rated important, while several new QoL aspects were brought up. Modifications in wording and phrasing led to two questionnaires with 77 items regarding general QoL aspects and 20 items regarding medical care. Important QoL aspects of PNH/AA patients are inappropriately captured with available QoL tools. Developing a new QoL questionnaire specific for this patient group is warranted.
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Drug therapy in pregnancy: the lessons of diethylstilbestrol, thalidomide, and bendectin.
Saunders, E J; Saunders, J A
1990-01-01
A pregnant woman and the fetus she carries face health risks from many sources. One risk that requires ongoing vigilance is the use of prescription drugs during pregnancy. The international health care community has been sensitized to the risks of drug use during pregnancy because of three pharmaceuticals that have caused widespread crises for mothers and their offspring. The crises that diethylstilbestrol, thalidomide, and, to a lesser extent, Bendectin have created in the past four decades are reviewed in this article. The lessons these drug crises can teach us are articulated in the belief that similar crises may be averted in the future.
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Treating Families of Bone Marrow Recipients and Donors
ERIC Educational Resources Information Center
Cohen, Marie; And Others
1977-01-01
Luekemia and aplastic anemia are beginning to be treated by bone marrow transplants, involving donors and recipients from the same family. Such intimate involvement in the patient's life and death struggles typically produces a family crisis and frequent maladaptive responses by various family members. (Author)
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Transfusion-Associated Microchimerism in Combat Casualties
2007-10-01
Hathaway WE, Brangle RW, Nelson TL, Roeckel IE. Aplastic anemia and alyphocytosis in an infant with hypogammaglobulinemia: graft-versus-host...multiple organ failure. Arch Surg. 1997; 132:620–624. 32. Dunne JR, Malone DL, Tracy JK, et al. Perioperative anemia : an independent risk factor for
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Clinical significance of acquired somatic mutations in aplastic anaemia.
Marsh, J C W; Mufti, G J
2016-08-01
Aplastic anaemia (AA) is frequently associated with other disorders of clonal haemopoiesis such as paroxysmal nocturnal haemoglobinuria (PNH), myelodysplastic syndrome (MDS) and T-large granular lymphocytosis. Certain clones may escape the immune attack within the bone marrow environment and proliferate and attain a survival advantage over normal haemopoietic stem cells, such as trisomy 8, loss of heterozygosity of short arm of chromosome 6 and del13q clones. Recently acquired somatic mutations (SM), excluding PNH clones, have been reported in around 20-25 % of patients with AA, which predispose to a higher risk of later malignant transformation to MDS/acute myeloid leukaemia. Furthermore, certain SM, such as ASXL1 and DNMT3A are associated with poor survival following immunosuppressive therapy, whereas PIGA, BCOR/BCORL1 predict for good response and survival. Further detailed and serial analysis of the immune signature in AA is needed to understand the pathogenetic basis for the presence of clones with SM in a significant proportion of patients.
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Nutritional status survey of aplastic anemia patients--a single center experience in China.
Li, Xinli; Feng, Yanyan; Wang, Hongyan; Song, Meijuan; Jin, Jingjing; Cui, Zhenzhu; Zheng, Yizhou
2016-05-01
To analyze the nutritional status of aplastic anemia (AA) patients. The nutrition-related anthropometric indicators and blood biochemical index of 622 newly-diagnosed AA patients were retrospectively analyzed. Of the cohort of AA patients, body mass index of non-severe AA (NSAA) patients were higher than those of severe AA (SAA) (p<0.05). The serum total protein and albumin protein levels of SAA patients differed from those of NSAA, and lower hemoglobin was correlated with lower serum albumin protein concentration (p<0.01). The concentration of B vitamins (folic acid and vitamin B12) of urban patients significantly differed from rural ones (P<0.01). Of the 97 cases of iron overload (15.6% of the entire patient group), the iron overload rate of SAA patients (19.1%) was much higher than that of NSAA (8.1%). AA patients exhibited malnutrition conditions; it would be helpful to conduct individualized dietary guidance and health education for patients. Copyright © 2015 Elsevier Inc. All rights reserved.
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Fluoxymesterone-induced gynaecomastia in a patient with childhood aplastic anaemia.
Lo, Tom Edward Ngo; Andal, Zillien C; Lantion-Ang, Frances Lina
2015-05-06
Gynaecomastia is a benign condition characterised by enlargement of the male breast. Drug-induced gynaecomastia merits deep consideration as it may account for as many as 25% of all cases of gynaecomastia in adults. Although the mechanism is not fully clear, some mechanisms include oestrogen-like activities, stimulation of testicular production of oestrogens, inhibition of testosterone synthesis or blockade of androgen action. Anabolic steroids, in particular when used during the pubertal stage, may cause significant irreversible gynaecomastia. We report a case of 28-year-old Filipino man with persistent gynaecomastia from fluoxymesterone used for aplastic anaemia during his prepubertal stage. Hormonal work ups for gynaecomastia all turned out normal, thus isolating the drug as the cause. The patient was unable to undergo breast reconstruction surgery due to haematological contraindications, but eventually referred to psychiatry for counselling. This case will highlight the paradoxical effect of androgenic steroid used during childhood on male breast proliferation during puberty. 2015 BMJ Publishing Group Ltd.
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Tutelman, Perri R; Aubert, Geraldine; Milner, Ruth A; Dalal, Bakul I; Schultz, Kirk R; Deyell, Rebecca J
2014-03-01
The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) cells and leucocyte subset telomere lengths in paediatric aplastic anaemia (AA) is unknown. Among 22 children receiving immunosuppressive therapy (IST) for AA, 73% (16/22) were PNH(pos) , of whom 94% achieved at least a partial response (PR) to IST; 11/16 (69%) achieved complete response (CR). Only 2/6 (33%) PNH(neg) patients achieved PR. PNH(pos) patients were less likely to fail IST compared to PNH(neg) patients (odds ratio 0·033; 95% confidence interval 0·002-0·468; P = 0·012). Children with AA had short granulocyte (P = 7·8 × 10(-9) ), natural killer cell (P = 6·0 × 10(-4) ), naïve T lymphocyte (P = 0·002) and B lymphocyte (P = 0·005) telomeres compared to age-matched normative data. © 2013 John Wiley & Sons Ltd.
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Resolution of psychosocial crises associated with flying in space
NASA Astrophysics Data System (ADS)
Suedfeld, Peter; Brcic, Jelena
2011-07-01
Erikson (1959) proposed a theoretical basis for healthy psychosocial development. His theory posits eight critical conflict situations throughout one's lifetime, each of which can result in a favorable or unfavorable resolution. Autobiographies, memoirs, interviews, personal diaries, and oral histories of 97 international astronauts were content analyzed to assess reported resolutions of Erikson's psychosocial crises, regardless of chronological sequence. We made comparisons across flight phases (before, during, and after), gender, nationality of home space agency, and flight duration. Astronauts reported more favorable than unfavorable outcomes across flight phases and demographic variables. Differences across demographic variables and flight phases, as well as the changes as a result of the flight are discussed.
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Unraveling hidden order in the dynamics of developed and emerging markets.
Berman, Yonatan; Shapira, Yoash; Ben-Jacob, Eshel
2014-01-01
The characterization of asset price returns is an important subject in modern finance. Traditionally, the dynamics of stock returns are assumed to lack any temporal order. Here we present an analysis of the autocovariance of stock market indices and unravel temporal order in several major stock markets. We also demonstrate a fundamental difference between developed and emerging markets in the past decade - emerging markets are marked by positive order in contrast to developed markets whose dynamics are marked by weakly negative order. In addition, the reaction to financial crises was found to be reversed among developed and emerging markets, presenting large positive/negative autocovariance spikes following the onset of these crises. Notably, the Chinese market shows neutral or no order while being regarded as an emerging market. These findings show that despite the coupling between international markets and global trading, major differences exist between different markets, and demonstrate that the autocovariance of markets is correlated with their stability, as well as with their state of development.
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Unraveling Hidden Order in the Dynamics of Developed and Emerging Markets
Berman, Yonatan; Shapira, Yoash; Ben-Jacob, Eshel
2014-01-01
The characterization of asset price returns is an important subject in modern finance. Traditionally, the dynamics of stock returns are assumed to lack any temporal order. Here we present an analysis of the autocovariance of stock market indices and unravel temporal order in several major stock markets. We also demonstrate a fundamental difference between developed and emerging markets in the past decade - emerging markets are marked by positive order in contrast to developed markets whose dynamics are marked by weakly negative order. In addition, the reaction to financial crises was found to be reversed among developed and emerging markets, presenting large positive/negative autocovariance spikes following the onset of these crises. Notably, the Chinese market shows neutral or no order while being regarded as an emerging market. These findings show that despite the coupling between international markets and global trading, major differences exist between different markets, and demonstrate that the autocovariance of markets is correlated with their stability, as well as with their state of development. PMID:25383630
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Li, Hongbo; Wang, Lin; Pang, Yan; Jiang, Zujun; Liu, Zenghui; Xiao, Haowen; Chen, Haijia; Ge, Xiaohu; Lan, Hai; Xiao, Yang
2017-02-14
The standard treatment for aplastic anemia (AA) in young patients is a matched sibling hematopoietic stem cell transplant. Transfusion of a chronic AA patient with allogeneic bone marrow-derived mesenchymal stromal cells (BMMSCs) is currently being developed as a cell-based therapy, and the safety and efficacy of such transfusions are being continuously improved. Nevertheless, the mechanisms by which BMMSCs exert their therapeutic effects remain to be elucidated. In this study, mesenchymal stromal cells (MSCs) obtained from bone marrow donors were concentrated and intravenously injected into 15 chronic AA patients who had been refractory to prior immunosuppressive therapy. We showed that BMMSCs modulate the levels of Th1, Th2, Th17 and Treg cells, as well as their related cytokines in chronic AA patients. Furthermore, the percentages of Th1 and Th17 cells among the H-MSCs decreased significantly, while the percentage Treg cells increased. The Notch/RBP-J/FOXP3/RORγt pathway was involved in modulating the Treg/Th17 balance after MSCs were transfused in vitro. Additionally, the role played by transfused MSCs in regulating the Treg/Th17 balance via the Notch/RBP-J/FOXP3/RORγt pathway was further confirmed in an AA mouse model. In summary, in humans with chronic AA, BMMSCs regulate the Treg/Th17 balance by affecting the Notch/RBP-J/FOXP3/RORγt pathway.
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Chao, Yu-Hua; Lin, Chiao-Wen; Pan, Hui-Hsien; Yang, Shun-Fa; Weng, Te-Fu; Peng, Ching-Tien; Wu, Kang-Hsi
2018-06-05
Although immune-mediated pathogenesis is considered an important aspect of severe aplastic anemia (SAA), its underlying mechanisms remain unclear. Mesenchymal stem cells (MSCs) are essential to the formation of specialized microenvironments in the bone marrow (BM), and MSC insufficiency can trigger the development of SAA. To find MSC alterations in the SAA BM, we compared BM MSCs from five children with SAA and five controls. Peripheral blood mononuclear cells (PBMCs) were cocultured with MSCs to evaluate the supportive effects of MSCs on hematopoiesis. Cytometric bead array immunoassay was used to determine cytokine excretion by MSCs. The immune functions of MSCs and their conditioned medium (CM) were evaluated by PBMC proliferation assays. SAA MSCs were characterized by a high percentage of cells in the abnormal sub-G1 phase of the cell cycle, which suggests an increased rate of apoptosis in SAA MSCs. In comparison with control MSCs, PBMCs cocultured with SAA MSCs displayed significantly reduced PBMC proliferation (P = 0.009). Aberrant cytokine profiles were secreted by SAA MSCs, with increased concentrations of interleukin-6, interferon-γ, tumor necrosis factor-α, and interleukin-1β in the CM. PBMC proliferation assays demonstrated additional immunosuppressive effects of SAA MSCs (P = 0.016) and their CM (P = 0.013). Our data revealed increased apoptosis and PBMC suppression of SAA MSCs. The alterations of MSCs may contribute to the formation of functionally abnormal microenvironments in SAA BM. © 2018 Wiley Periodicals, Inc.
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Lee, Sung-Eun; Park, Sung Soo; Jeon, Young-Woo; Yoon, Jae-Ho; Cho, Byung-Sik; Eom, Ki-Sung; Kim, Yoo-Jin; Lee, Seok; Min, Chang-Ki; Kim, Hee-Je; Cho, Seok-Goo; Kim, Dong-Wook; Min, Woo-Sung; Lee, Jong Wook
2017-10-01
The aim of this study was to evaluate the long-term outcomes of allogeneic stem cell transplantation (SCT) in patients with paroxysmal nocturnal hemoglobinuria (PNH) with or without aplastic anemia (AA). A total of 33 patients with PNH clones who underwent allogeneic SCT were analyzed. After a median follow-up of 57 months (range, 6.0-151.3), the 5-year estimated overall survival rate was 87.9±5.7%. Four patients died of transplant-related mortality (TRM). With the exception of one patient with early TRM, 32 patients were engrafted. Two patients who had developed delayed GF received a second transplant and recovered. The cumulative incidences of acute graft-vs-host disease (GVHD) (≥grade II) and chronic GVHD (≥moderate) were 27.3±7.9% and 18.7±7.0%, respectively. Twenty-one patients receiving SCT with reduced-intensity conditioning (RIC) had available follow-up data for PNH cell population for the first 6 months post-transplant. Analysis of these data revealed that the PNH clones disappeared within approximately 2 months. RIC regimen was sufficient to eradicate PNH clones with sustained donor-type engraftment after allogeneic SCT. Therefore, application of allogeneic SCT with RIC should be considered in patients with PNH, in accordance with the severity of the underlying bone marrow failure. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
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Corporate Psychology: An Answer to Occupational Mental Health.
ERIC Educational Resources Information Center
Leonards, Jeffrey T.
1981-01-01
Studies corporate in-house, human resource programs. Suggests these programs provide psychological assistance for problems considered to be normal episodes of human development. Proposes most of the developmental crises encountered by the corporate psychologist are associated with stress, midlife, or career development. (Author)
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Organizational Development Interventions for Enhancing Creativity in the Workplace.
ERIC Educational Resources Information Center
Basadur, Min
1997-01-01
Evaluates traditional organizational development approaches to crises in commitment and adaptability, and presents a new approach to organizational development based on organizational creativity. Discusses the need to encourage employees to master new thinking skills and create an infrastructure that ensures these skills will be used regularly.…
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ERIC Educational Resources Information Center
Koster, Francis
The paper presents a review of literature on warnings of social crises and applies the literature to selected crises predicted in America's future. It is intended for use by futurists and policy makers as they devise ways to help society cope with crises. Warnings are interpreted as combining statement of a problem with proposal of a course of…
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ERIC Educational Resources Information Center
Burgin, Rick A.
2012-01-01
Large-scale crises continue to surprise, overwhelm, and shatter college and university campuses. While the devastation to physical plants and persons is often evident and is addressed with crisis management plans, the number of emotional casualties left in the wake of these large-scale crises may not be apparent and are often not addressed with…
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Parvovirus B19 infection as a cause of acute myositis in an adult.
Cakirca, Mustafa; Karatoprak, Cumali; Ugurlu, Serdal; Zorlu, Mehmet; Kıskaç, Muharrem; Çetin, Güven
2015-01-01
Parvovirus B19 infection is often asymptomatic, but clinical expressions may include transient aplastic crisis, erythema infectiosum, non-immune hydrops fetalis, and chronic red cell aplasia. This virus has also been associated with rheumatoid arthritis and other autoimmune connective tissue diseases; however, we could not identify any acute adult myositis case developed after a Parvovirus B19 infection in the literature. For this reason, we would like to present a rare case of acute myositis developed after Parvovirus B19 infection. In patients presenting with symptoms of fever, rash on the legs and myositis, viral infections such as Parvovirus B19 should be kept in mind. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.
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46 CFR Appendix C to Subpart C of... - Medical Surveillance Guidelines for Benzene
Code of Federal Regulations, 2014 CFR
2014-10-01
... depression of the hematopoietic system, pancytopenia, aplastic anemia, and leukemia. Inhalation of high concentrations may affect the functioning of the central nervous system. Aspiration of small amounts of liquid... an initial stimulatory effect on the central nervous system characterized by exhilaration, nervous...
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46 CFR Appendix C to Subpart C to... - Medical Surveillance Guidelines for Benzene
Code of Federal Regulations, 2011 CFR
2011-10-01
... depression of the hematopoietic system, pancytopenia, aplastic anemia, and leukemia. Inhalation of high concentrations may affect the functioning of the central nervous system. Aspiration of small amounts of liquid... an initial stimulatory effect on the central nervous system characterized by exhilaration, nervous...
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46 CFR Appendix C to Subpart C of... - Medical Surveillance Guidelines for Benzene
Code of Federal Regulations, 2013 CFR
2013-10-01
... depression of the hematopoietic system, pancytopenia, aplastic anemia, and leukemia. Inhalation of high concentrations may affect the functioning of the central nervous system. Aspiration of small amounts of liquid... an initial stimulatory effect on the central nervous system characterized by exhilaration, nervous...
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46 CFR Appendix C to Subpart C of... - Medical Surveillance Guidelines for Benzene
Code of Federal Regulations, 2012 CFR
2012-10-01
... depression of the hematopoietic system, pancytopenia, aplastic anemia, and leukemia. Inhalation of high concentrations may affect the functioning of the central nervous system. Aspiration of small amounts of liquid... an initial stimulatory effect on the central nervous system characterized by exhilaration, nervous...
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Dixit, Ramakant; Dixit, Kalpana; Paramez, A. R.
2010-01-01
Cleidocranial dysplasia is a rare autosomal dominant condition with generalized dysplasia of bone, characterized by delayed closer of cranial sutures, hypoplastic or aplastic clavicles, short stature, dental abnormalities and a variety of other skeletal abnormalities. We present a seven-year-old female child presenting with classical features of cleidocranial dysplasia. PMID:20931042
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Foreign exchange rate entropy evolution during financial crises
NASA Astrophysics Data System (ADS)
Stosic, Darko; Stosic, Dusan; Ludermir, Teresa; de Oliveira, Wilson; Stosic, Tatijana
2016-05-01
This paper examines the effects of financial crises on foreign exchange (FX) markets, where entropy evolution is measured for different exchange rates, using the time-dependent block entropy method. Empirical results suggest that financial crises are associated with significant increase of exchange rate entropy, reflecting instability in FX market dynamics. In accordance with phenomenological expectations, it is found that FX markets with large liquidity and large trading volume are more inert - they recover quicker from a crisis than markets with small liquidity and small trading volume. Moreover, our numerical analysis shows that periods of economic uncertainty are preceded by periods of low entropy values, which may serve as a tool for anticipating the onset of financial crises.
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[Management of hyperglycemic crises and severe hypoglycemia in the emergency department].
Miyake, Yasufumi
2014-02-01
The morbidity of hyperglycemic crises and acute hypoglycemic attacks in patients with diabetes mellitus has been increasing for the past several decades. One of the reasons for this is the increase in the number of patients with diabetes. The increased proportion of aging and isolation in society is another reason. The author has discussed patients with these complaints: their epidemiology, pathophysiology, and management in the emergency department. Hyperglycemic crises include diabetic ketoacidosis (DKA), hyperosmolar hyperglycemic syndrome (HHS), and lactic acidosis (LA). Younger patients with type 1 diabetes suffer from DKA; inappropriate insulin therapy or infection is usually the trigger. Older patients with type 2 diabetes are at risk of HHS in the course of sepsis or in the perioperative period. The treatment of both types of patients is common. Sufficient amount of intravenous extracellular fluid and constant infusion of insulin are essential. The development of LA is not associated with the use of metformin, but with the severity of the pre-existing disease. Early recognition and aggressive treatment is vital to improving the prognosis of hyperglycemic emergencies and severe hypoglycemic episodes.
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Greek-Turkish Crises since 1955. Implications for Greek-Turkish Conflict Management
1997-03-01
NAVAL POSTGRADUATE SCHOOL MONTEREY, CALIFORNIA THESIS GREEK-TURKISH CRISES SINCE 1955. IMPLICATIONS FOR GREEK-TURKISH CONFLICT MANAGEMENT by...EU, WEU) have only to gain from a Greek-Turkish rapprochement. 14. SUBJECT TERMS GrEek-Turkish RElATiONS, CRiSiS MANAgEMENT, CONfLICT management 15...crises, because the intended outcome of mediation attempts has been regional stability instead of Greek-Turkish conflict management . Power mediation
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Scenarios for Evolving Seismic Crises: Possible Communication Strategies
NASA Astrophysics Data System (ADS)
Steacy, S.
2015-12-01
Recent advances in operational earthquake forecasting mean that we are very close to being able to confidently compute changes in earthquake probability as seismic crises develop. For instance, we now have statistical models such as ETAS and STEP which demonstrate considerable skill in forecasting earthquake rates and recent advances in Coulomb based models are also showing much promise. Communicating changes in earthquake probability is likely be very difficult, however, as the absolute probability of a damaging event is likely to remain quite small despite a significant increase in the relative value. Here, we use a hybrid Coulomb/statistical model to compute probability changes for a series of earthquake scenarios in New Zealand. We discuss the strengths and limitations of the forecasts and suggest a number of possible mechanisms that might be used to communicate results in an actual developing seismic crisis.
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Evo-devo of infantile and childhood growth.
Hochberg, Ze'ev; Albertsson-Wikland, Kerstin
2008-07-01
Human size is a tradeoff between the evolutionary advantages and disadvantages of being small or big. We now propose that adult size is determined to an important extent during transition from infancy to childhood. This transition is marked by a growth spurt. A delay in the transition has a lifelong impact on stature and is responsible for 44% of children with short stature in developed countries and many more in developing countries. Here, we present the data and theory of an evolutionary adaptive strategy of plasticity in the timing of transition from infancy into childhood to match the prevailing energy supply. We propose that humans have evolved to withstand energy crises by decreasing their body size, and that evolutionary short-term adaptations to energy crises trigger a predictive adaptive response that modify the transition into childhood, culminating in short stature.
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Leuschner, Vincenz; Fiedler, Nora; Schultze, Martin; Ahlig, Nadine; Göbel, Kristin; Sommer, Friederike; Scholl, Johanna; Cornell, Dewey; Scheithauer, Herbert
2017-01-01
The standardized, indicated school-based prevention program "Networks Against School Shootings" combines a threat assessment approach with a general model of prevention of emergency situations in schools through early intervention in student psychosocial crises and training teachers to recognize warning signs of targeted school violence. An evaluation study in 98 German schools with 3,473 school staff participants (M age = 46.2 years) used a quasi-experimental comparison group design with three measurement points (pre, post, and 7 months followup) with schools randomly allocated to implementation conditions. The study found increases in teachers' expertise and evaluation skills, enhanced abilities to identify students experiencing a psychosocial crisis, and positive secondary effects (e.g., teacher-student interaction, feelings of safety). © 2017 The Authors. Child Development © 2017 Society for Research in Child Development, Inc.
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Science during crisis: the application of social science during major environmental crises
Machlis, Gary; Ludwig, Kris; Manfredo, Michael J.; Vaske, Jerry J.; Rechkemmer, Andreas; Duke, Esther
2014-01-01
Historical and contemporary experience suggests that science plays an increasingly critical role in governmental and institutional responses to major environmental crises. Recent examples include major western wildfires (2009), the Deepwater Horizon oil spill (2010), the Fukushima nuclear accident (2011), and Hurricane Sandy (2012). The application of science during such crises has several distinctive characteristics, as well as essential requirements if it is to be useful to decision makers. these include scope conditions that include coupled natural/human systems, clear statement of uncertainties and limitations, description of cascading consequences, accurate sense of place, estimates of magnitude of impacts, identification of beneficiaries and those adversely affected, clarity and conciseness, compelling visualization and presentation, capacity to speak "truth to power", and direct access to decision makers. In this chapter, we explore the role and significance of science – including all relevant disciplines and focusing attention on the social sciences – in responding to major environmental crises. We explore several important questions: How is science during crisis distinctive? What social science is most useful during crises? What distinctive characteristics are necessary for social science to make meaningful contributions to emergency response and recovery? How might the social sciences be integrated into the strategic science needed to respond to future crises? The authors, both members of the Department of the Interior's innovative Strategic Sciences Group, describe broad principles of engagement as well as specific examples drawn from history, contemporary efforts (such as during the Deepwater Horizon oil spill), and predictions of environmental crises still to be confronted.
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NASA Astrophysics Data System (ADS)
Larsen, Tulinda Deegan
In this study the researcher provides a behavioral framework for managing massive airline flight disruptions (MAFD) in the United States. Under conditions of MAFD, multiple flights are disrupted throughout the airline's route network, customer service is negatively affected, additional costs are created for airlines, and governments intervene. This study is different from other studies relating to MAFD that have focused on the operational, technical, economic, financial, and customer service impacts. The researcher argues that airlines could improve the management of events that led to MAFD by applying the principles of crisis management where the entire organization is mobilized, rather than one department, adapting organization development (OD) interventions to implement change and organization learning (OL) processes to create culture of innovation, resulting in sustainable improvement in customer service, cost reductions, and mitigation of government intervention. At the intersection of crisis management, OD, and OL, the researcher has developed a new conceptual framework that enhances the resiliency of individuals and organizations in responding to unexpected-yet-recurring crises (e.g., MAFD) that impact operations. The researcher has adapted and augmented Lalonde's framework for managing crises through OD interventions by including OL processes. The OD interventions, coupled with OL, provide a framework for airline leaders to manage more effectively events that result in MAFD with the goal of improving passenger satisfaction, reducing costs, and preventing further government intervention. Further research is warranted to apply this conceptual framework to unexpected-yet-recurring crises that affect operations in other industries.
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Morphological Manifestations of Parvovirus B19 Infection in the Bone Marrow.
1997-01-09
Parvovirus B19 (PV B19 ) preferentially infects erythroid progenitor cells in the bone marrow, frequently causing anemia along with transient aplastic...infection. We devised a highly sensitive two-round, nested PCR procedure to detect PV B19 . Eight of 78 clinical specimens from individuals with
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2017-07-13
Bone Marrow Failure Syndrome; Severe Aplastic Anemia; Severe Congenital Neutropenia; Amegakaryocytic Thrombocytopenia; Diamond-Blackfan Anemia; Schwachman Diamond Syndrome; Primary Immunodeficiency Syndromes; Acquired Immunodeficiency Syndromes; Histiocytic Syndrome; Familial Hemophagocytic Lymphocytosis; Lymphohistiocytosis; Macrophage Activation Syndrome; Langerhans Cell Histiocytosis (LCH); Hemoglobinopathies; Sickle Cell Disease; Sickle Cell-beta-thalassemia
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Use of verbal autopsy and social autopsy in humanitarian crises
Balabanova, Dina
2018-01-01
Introduction Two billion people live in countries affected by conflict, violence and fragility. These are exceptional situations in which mortality shifts dramatically and in which civil registration and vital statistics systems are often weakened or cease to function. Verbal autopsy and social autopsy (VA and SA) are methods used to assign causes of death and understand the contexts in which these occur, in settings where information is otherwise unavailable. This review sought to explore the use of VA and SA in humanitarian crises, with a focus on how these approaches are used to inform policy and programme responses. Methods A rapid scoping review was conducted on the use of VA and SA in humanitarian crises in low and middle-income countries since 1991. Drawing on a maximum variation approach, two settings of application (‘application contexts’) were selected and investigated via nine semi-structured expert interviews. Results VA can determine causes of death in crisis-affected populations where no other registration system is in place. Combined with SA and active community involvement, these methods can deliver a holistic view of obstacles to seeking and receiving essential healthcare, yielding context-specific information to inform appropriate responses. The contexts in which VA and SA are used require adaptations to standard tools, and new mobile developments in VA raise specific ethical considerations. Furthermore, collecting and sythesising data in a timely, continuous manner, and ensuring coordination and communication between agencies, is important to realise the potential of these approaches. Conclusion VA and SA are valuable research methods to foster evidence-informed responses for populations affected by humanitarian crises. When coordinated and communicated effectively, data generated through these methods can help to identify levels, causes and circumstances of deaths among vulnerable groups, and can enable planning and allocating resources effectively, potentially improving health system resilience to future crises. PMID:29736275
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Anderlini, Paolo; Wu, Juan; Gersten, Iris; Ewell, Marian; Tolar, Jakob; Antin, Joseph H; Adams, Roberta; Arai, Sally; Eames, Gretchen; Horwitz, Mitchell E; McCarty, John; Nakamura, Ryotaro; Pulsipher, Michael A; Rowley, Scott; Leifer, Eric; Carter, Shelly L; DiFronzo, Nancy L; Horowitz, Mary M; Confer, Dennis; Deeg, H Joachim; Eapen, Mary
2015-09-01
The optimum preparative regimen for unrelated donor marrow transplantation in patients with severe aplastic anaemia remains to be established. We investigated whether the combination of fludarabine, anti-thymocyte globulin, and total body irradiation (TBI) would enable reduction of the cyclophosphamide dose to less than 200 mg/kg while maintaining engraftment and having a survival similar to or better than that with standard regimens using a cyclophosphamide dose of 200 mg/kg (known to be associated with significant organ toxicity) for unrelated donor transplantation for severe aplastic anaemia. We have previously shown that cyclophosphamide at 150 mg/kg resulted in excess toxicity and its omission (0 mg/kg) resulted in unacceptable graft failure (three of three patients had secondary graft failure). Here we report results for the 50 mg/kg and 100 mg/kg cohorts. In a multicentre phase 1-2 study, patients (aged ≤65 years) with severe aplastic anaemia, adequate organ function, and an unrelated adult marrow donor HLA matched at the allele level for HLA A, B, C, and DRB1 or mismatched at a single HLA locus received bone marrow grafts from unrelated donors. All patients received anti-thymocyte globulin (rabbit derived 3 mg/kg per day, intravenously, on days -4 to -2, or equine derived 30 mg/kg per day, intravenously, on days -4 to -2), fludarabine (30 mg/m(2) per day, intravenously, on days -5 to -2), and TBI (2 Gy). Cyclophosphamide dosing started at 150 mg/kg and was de-escalated in steps of 50 mg/kg (to 100 mg/kg, 50 mg/kg, and 0 mg/kg). The primary endpoint was the selection of the optimum cyclophosphamide dose based on assessments of graft failure (primary or secondary), toxicity, and early death during 100 days of follow-up after the transplant; this is the planned final analysis for the primary endpoint. This trial is registered with ClinicalTrials.gov, number NCT00326417. 96 patients had bone marrow transplant. At day 100, 35 (92%) of 38 patients were engrafted and alive in the cyclophosphamide 50 mg/kg cohort and 35 (85%) of 41 in the 100 mg/kg cohort. Cyclophosphamide 50 mg/kg and 100 mg/kg resulted in posterior means for fatality without graft failure of 0·7% (credible interval 0-3·3) and 1·4% (0-4·9), respectively. Three patients (8%) had graft failure with cyclophosphamide 50 mg/kg and six (15%) with cyclophosphamide 100 mg/kg. Four (11%) patients had major regimen-related toxicity with cyclophosphamide 50 mg/kg and nine (22%) with cyclophosphamide 100 mg/kg. The most common organ toxicity was pulmonary (grade 3 or 4 dyspnoea or hypoxia including mechanical ventilation), and occurred in three (8%) and four (10%) patients given cyclophosphamide 50 mg/kg and 100 mg/kg, respectively. Cyclophosphamide at 50 mg/kg and 100 mg/kg with TBI 2 Gy, fludarabine, and anti-thymocyte globulin results in effective conditioning and few early deaths after unrelated donor transplantation for severe aplastic anaemia. These doses of cyclophosphamide provide a framework for further regimen optimisation strategies. US National Heart, Lung, and Blood Institute and National Cancer Institute. Copyright © 2015 Elsevier Ltd. All rights reserved.
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Purev, Enkhtsetseg; Tian, Xin; Aue, Georg; Pantin, Jeremy; Vo, Phuong; Shalabi, Reem; Reger, Robert N; Cook, Lisa; Ramos, Catalina; Cho, Elena; Worthy, Tat'yana; Khuu, Hanh; Stroncek, David; Young, Neal S; Childs, Richard W
2017-03-01
Allogeneic haematopoietic stem cell transplantation is curative for severe aplastic anaemia (SAA) unresponsive to immunosuppressive therapy. To reduce chronic graft-versus-host disease (GVHD), which occurs more frequently after peripheral blood stem cell (PBSC) transplantation compared to bone-marrow transplantation (BMT), and to prevent graft rejection, we developed a novel partial T-cell depleted transplant that infuses high numbers of granulocyte colony-stimulating factor-mobilized CD34 + selected PBSCs combined with a BMT-equivalent dose of non-mobilized donor T-cells. Fifteen patients with refractory SAA received cyclophosphamide, anti-thymocyte globulin and fludarabine conditioning, and were transplanted with a median 8 × 10 6 CD34 + cells/kg and 2 × 10 7 non-mobilized CD3 + T-cells/kg from human leucocyte antigen-matched sibling donors. All achieved sustained engraftment with only two developing acute and two developing chronic GVHD. With a 3·5-year median follow-up, 86% of patients survived and were transfusion-independent. When compared to a retrospective cohort of 56 bone-marrow failure patients that received the identical transplant preparative regimen and GVHD prophylaxis with the exception that the allograft contained unmanipulated PBSCs, partial T-cell depleted transplant recipients had delayed donor T-cell chimerism and relative reduction of 75% in the incidence of acute grade II-IV GVHD (13% vs. 52%; P = 0·010) and of 82% in chronic GVHD (13% vs. 72%; P = 0·0004). In multivariate analysis, partial T-cell depleted transplants remained significantly associated with a reduced risk of GVHD. In conclusion, for patients with refractory SAA, this novel transplant strategy achieves excellent engraftment and survival when compared to unmanipulated PBSC transplants and dramatically reduces the incidence of both acute and chronic GVHD. © 2017 John Wiley & Sons Ltd.
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Goldhaber-Fiebert, Sara N; Macrae, Carl
2018-03-01
How can teams manage critical events more effectively? There are commonly gaps in performance during perioperative crises, and emergency manuals are recently available tools that can improve team performance under stress, via multiple mechanisms. This article examines how the principles of implementation science and quality improvement were applied by multiple teams in the development, testing, and systematic implementations of emergency manuals in perioperative care. The core principles of implementation have relevance for future patient safety innovations perioperatively and beyond, and the concepts of emergency manuals and interprofessional teamwork are applicable for diverse fields throughout health care. Copyright © 2017 Sara N. Goldhaber-Fiebert, Carl Macrae. Published by Elsevier Inc. All rights reserved.
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Daly, G.G.; Mayor, T.H.
This paper develops a technique for extracting the expectations embedded in the current prices of energy-using durable goods and applies it to be used-car markets during the two energy crises of the 1970s. The resulting estimates indicate that consumers took the energy crises seriously and formed expectations about future gasoline prices that appear rational when compared with the historical gasoline price series, with the forecasts of specialists and experts, or with the actual postsample behavior of gasoline prices. The evidence therefore supports the view that consumers are able to make rather complex choices with a great deal of rationality andmore » casts doubt on the wisdom of policies based on assumptions to the contrary. 17 references, 2 figures.« less
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Economy Over Security: Why Crises Fail to Impact Economic Behavior in East Asia
2017-12-01
SECURITY: WHY CRISES FAIL TO IMPACT ECONOMIC BEHAVIOR IN EAST ASIA by Aaron R. Sipos December 2017 Thesis Advisor: Michael Glosny Second...REPORT TYPE AND DATES COVERED Master’s thesis 4. TITLE AND SUBTITLE ECONOMY OVER SECURITY: WHY CRISES FAIL TO IMPACT ECONOMIC BEHAVIOR IN EAST...release. Distribution is unlimited. 12b. DISTRIBUTION CODE 13. ABSTRACT (maximum 200 words) This study examines changes in economic behavior in
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A half-baked solution: drivers of water crises in Mexico
NASA Astrophysics Data System (ADS)
Godinez Madrigal, Jonatan; van der Zaag, Pieter; van Cauwenbergh, Nora
2018-02-01
Mexico is considered a regional economic and political powerhouse because of the size of its economy, and a large population in constant growth. However, this same growth accompanied by management and governance failures are causing several water crises across the country. The paper aims at identifying and analyzing the drivers of water crises. Water authorities seem to focus solely on large infrastructural schemes to counter the looming water crises, but fail to structure a set of policies for the improvement of management and governance institutions. The paper concludes with the implications of a business-as-usual policy based on infrastructure for solving water problems, which include a non-compliance to the human right to water and sanitation, ecosystem collapses and water conflicts.
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Time series analysis of S&P 500 index: A horizontal visibility graph approach
NASA Astrophysics Data System (ADS)
Vamvakaris, Michail D.; Pantelous, Athanasios A.; Zuev, Konstantin M.
2018-05-01
The behavior of stock prices has been thoroughly studied throughout the last century, and contradictory results have been reported in the corresponding literature. In this paper, a network theoretical approach is provided to investigate how crises affected the behavior of US stock prices. We analyze high frequency data from S&P500 via the Horizontal Visibility Graph method, and find that all major crises that took place worldwide in the last twenty years, affected significantly the behavior of the price-index. Nevertheless, we observe that each of those crises impacted the index in a different way and magnitude. Interestingly, our results suggest that the predictability of the price-index series increases during the periods of crises.
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Pagán, Josué; Risco-Martín, José L; Moya, José M; Ayala, José L
2016-08-01
Prediction of symptomatic crises in chronic diseases allows to take decisions before the symptoms occur, such as the intake of drugs to avoid the symptoms or the activation of medical alarms. The prediction horizon is in this case an important parameter in order to fulfill the pharmacokinetics of medications, or the time response of medical services. This paper presents a study about the prediction limits of a chronic disease with symptomatic crises: the migraine. For that purpose, this work develops a methodology to build predictive migraine models and to improve these predictions beyond the limits of the initial models. The maximum prediction horizon is analyzed, and its dependency on the selected features is studied. A strategy for model selection is proposed to tackle the trade off between conservative but robust predictive models, with respect to less accurate predictions with higher horizons. The obtained results show a prediction horizon close to 40min, which is in the time range of the drug pharmacokinetics. Experiments have been performed in a realistic scenario where input data have been acquired in an ambulatory clinical study by the deployment of a non-intrusive Wireless Body Sensor Network. Our results provide an effective methodology for the selection of the future horizon in the development of prediction algorithms for diseases experiencing symptomatic crises. Copyright © 2016 Elsevier Inc. All rights reserved.
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How do economic crises affect migrants' risk of infectious disease? A systematic-narrative review.
Kentikelenis, Alexander; Karanikolos, Marina; Williams, Gemma; Mladovsky, Philipa; King, Lawrence; Pharris, Anastasia; Suk, Jonathan E; Hatzakis, Angelos; McKee, Martin; Noori, Teymur; Stuckler, David
2015-12-01
It is not well understood how economic crises affect infectious disease incidence and prevalence, particularly among vulnerable groups. Using a susceptible-infected-recovered framework, we systematically reviewed literature on the impact of the economic crises on infectious disease risks in migrants in Europe, focusing principally on HIV, TB, hepatitis and other STIs. We conducted two searches in PubMed/Medline, Web of Science, Cochrane Library, Google Scholar, websites of key organizations and grey literature to identify how economic changes affect migrant populations and infectious disease. We perform a narrative synthesis in order to map critical pathways and identify hypotheses for subsequent research. The systematic review on links between economic crises and migrant health identified 653 studies through database searching; only seven met the inclusion criteria. Fourteen items were identified through further searches. The systematic review on links between economic crises and infectious disease identified 480 studies through database searching; 19 met the inclusion criteria. Eight items were identified through further searches. The reviews show that migrant populations in Europe appear disproportionately at risk of specific infectious diseases, and that economic crises and subsequent responses have tended to exacerbate such risks. Recessions lead to unemployment, impoverishment and other risk factors that can be linked to the transmissibility of disease among migrants. Austerity measures that lead to cuts in prevention and treatment programmes further exacerbate infectious disease risks among migrants. Non-governmental health service providers occasionally stepped in to cater to specific populations that include migrants. There is evidence that migrants are especially vulnerable to infectious disease during economic crises. Ring-fenced funding of prevention programs, including screening and treatment, is important for addressing this vulnerability. © The Author 2015. Published by Oxford University Press on behalf of the European Public Health Association.
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How do economic crises affect migrants’ risk of infectious disease? A systematic-narrative review
Karanikolos, Marina; Williams, Gemma; Mladovsky, Philipa; King, Lawrence; Pharris, Anastasia; Suk, Jonathan E.; Hatzakis, Angelos; McKee, Martin; Noori, Teymur; Stuckler, David
2015-01-01
Background: It is not well understood how economic crises affect infectious disease incidence and prevalence, particularly among vulnerable groups. Using a susceptible-infected-recovered framework, we systematically reviewed literature on the impact of the economic crises on infectious disease risks in migrants in Europe, focusing principally on HIV, TB, hepatitis and other STIs. Methods: We conducted two searches in PubMed/Medline, Web of Science, Cochrane Library, Google Scholar, websites of key organizations and grey literature to identify how economic changes affect migrant populations and infectious disease. We perform a narrative synthesis in order to map critical pathways and identify hypotheses for subsequent research. Results: The systematic review on links between economic crises and migrant health identified 653 studies through database searching; only seven met the inclusion criteria. Fourteen items were identified through further searches. The systematic review on links between economic crises and infectious disease identified 480 studies through database searching; 19 met the inclusion criteria. Eight items were identified through further searches. The reviews show that migrant populations in Europe appear disproportionately at risk of specific infectious diseases, and that economic crises and subsequent responses have tended to exacerbate such risks. Recessions lead to unemployment, impoverishment and other risk factors that can be linked to the transmissibility of disease among migrants. Austerity measures that lead to cuts in prevention and treatment programmes further exacerbate infectious disease risks among migrants. Non-governmental health service providers occasionally stepped in to cater to specific populations that include migrants. Conclusions: There is evidence that migrants are especially vulnerable to infectious disease during economic crises. Ring-fenced funding of prevention programs, including screening and treatment, is important for addressing this vulnerability. PMID:26318852
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Crises Management in the Oil and Gas Industry: The Niger Delta Experience
NASA Astrophysics Data System (ADS)
Odemene, Glory C.
The Niger Delta crises escalated beyond the borders of the Nigerian nation to become an issue that affected individuals and corporations around the world. This study led to the discovery of how the local crises escalated with international implications. This discovery was accomplished by addressing how the Niger Delta crises escalated from villages to international scenes, with notable impacts on the environment, health, safety, security, and financial segments of local, international, private, and corporate entities. Using Sweeny's crisis decision theory and Lazarus and Folkman's coping theory, the study considered the coping strategies of community members, the decisions, and actions they took in response to the management approaches of the government and the oil and gas companies (OGCs). This qualitative study utilized historical narrative to collect data by interviewing 4 participants who lived and worked in the region during the crises. NVivo was used for manual and automatic coding of data, as well as for categorization and connection of codes. Content analysis of identified codes and categories revealed the themes and trends in the experiences narrated by participants. Findings include the root causes, trend of escalation, and management strategies of the government and the OGCs that influenced the crises. These findings will help to influence policies and practices in the region and enhance effective management of current and emerging conflicts, with possibilities of restoring stability and security in the areas and in the nation at large.
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An Investigation of Computer-based Simulations for School Crises Management.
ERIC Educational Resources Information Center
Degnan, Edward; Bozeman, William
2001-01-01
Describes development of a computer-based simulation program for training school personnel in crisis management. Addresses the data collection and analysis involved in developing a simulated event, the systems requirements for simulation, and a case study of application and use of the completed simulation. (Contains 21 references.) (Authors/PKP)
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Erik Erikson's Theory of Psychosocial Development and Vocational Behavior
ERIC Educational Resources Information Center
Munley, Patrick H.
1975-01-01
Stage resolution attitudes, derived from the first six stage crises outlined by Erikson, were explored as variables influencing problems in vocational choice and vocational maturity. Findings indicated students who made adjusted vocational choices and developed mature career attitudes had also been more successful resolving the first six…
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ERIC Educational Resources Information Center
Poston, John M.; Hanson, William E.; Schwiebert, Valerie
2012-01-01
The relationship between episodic and dispositional forgiveness and the resolution of Erikson's (1963) psychosocial crises were explored in this study. Participants (N = 66) completed the Enright Forgiveness Inventory (Enright & Rique, 2004), Tendency to Forgive Scale (Brown, 2003), and Measures of Psychosocial Development (Hawley, 1988). Results…
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The Role of Basic Education in Post-Conflict Recovery
ERIC Educational Resources Information Center
Barakat, Sultan; Connolly, David; Hardman, Frank; Sundaram, Vanita
2013-01-01
The last decade has seen a growing recognition amongst international donors, development agencies, non-government organisations and academics of the vital role education can play in bringing about recovery following violent conflict, natural disaster and other crises. This has led to the development of increasingly targeted and sophisticated…
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“Hitting the wall”: Lived experiences of mental health crises
Karlsson, Bengt; Lofthus, Ann-Mari; Davidson, Larry
2011-01-01
Background As Norway moves toward the provision of home-based crisis response, knowledge is needed about understandings of mental health crisis and effective ways of addressing crises within the home. Objective To elicit and learn from service users’ experiences about the subjective meanings of crisis and what kind of help will be most effective in resolving mental health crises. Theoretical A phenomenological-hermeneutic cooperative inquiry method was used to elicit and analyse focus group responses from mental health service users who had experienced crises. Results Findings clustered into three themes: (1) Crisis as multifaceted and varied experiences; (2) losing the skills and structure of everyday life; and (3) complexities involved in family support. Conclusion Several aspects of crises require an expansion of the biomedical model of acute intervention to include consideration of the personal and familial meaning of the crisis, attention to the home context, and activities of daily living that are disrupted by the crisis, and ways for the person and the family to share in and learn from resolution of the crisis. PMID:22140400
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Kear, E.
During the energy crises of the 1970s, commercial/office space lighting was an easy target for energy conservation. The first energy conservation measures consisted of turning off the lights when the building was closed for business, but this was not easy for some since many newer buildings were designed without convenient light switches or, for that matter, any switches. Alternative lighting technologies were quickly placed into service; they provided energy savings but usually at a loss of quality and quantity (such as the low-pressure sodium lamps). When the energy crises were over, lighting energy use rose again, but not to itsmore » pre-crises level: everyone had at least learned to turn out the lights at night. Eventually, improved lighting fixtures and lamps were developed, T-8s for example, which provide increased quality and quantity with decreased energy use, and now generally less energy is used to illuminate office and commercial spaces. So, since less energy is used for lighting, why is energy use in the commercial sector growing? One answer is the current explosion in the application of electronic office equipment technologies. Energy consumption by `information` equipment in the commercial segment, including large computer systems, has tripled in the last 10 years and could easily double in the next 10. While there are no driving crises as in the 1970s, there are still some lessons to be learned from lighting, such as: (1) Teaching everyone to turn off his/her PC when it`s not in use. This won`t ruin the hard drive, but it might make the computer last longer and it will save energy. (2) Encouraging the development of energy saving features, including Energy Star compliance. Fortunately, this is not too difficult, and is consistent with existing trends in the industry. (3) Teaching people to buy and use the energy-saving features. This could be harder than one might think, since some Energy Star-compliant devices still have a few `bugs.`« less
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Bone Marrow and Kidney Transplant for Patients With Chronic Kidney Disease and Blood Disorders
2017-03-21
Chronic Kidney Disease; Acute Myeloid Leukemia (AML); Acute Lymphoblastic Leukemia (ALL); Chronic Myelogenous Leukemia (CML); Chronic Lymphocytic Leukemia (CLL); Non-Hodgkin's Lymphoma (NHL); Hodgkin Disease; Multiple Myeloma; Myelodysplastic Syndrome (MDS); Aplastic Anemia; AL Amyloidosis; Diamond Blackfan Anemia; Myelofibrosis; Myeloproliferative Disease; Sickle Cell Anemia; Autoimmune Diseases; Thalassemia
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Abayomi, Akin; Gevao, Sahr; Conton, Brian; Deblasio, Pasquale; Katz, Rebecca
2016-01-01
This paper describes the formation of a civil society consortium, spurred to action by frustration over the Ebola crises, to facilitate the development of infrastructure and frameworks including policy development to support a harmonized, African approach to health crises on the continent. The Global Emerging Pathogens Treatment Consortium, or GET, is an important example of how African academics, scientists, clinicians and civil society have come together to initiate policy research, multilevel advocacy and implementation of initiatives aimed at building African capacity for timely and effective mitigations strategies against emerging infectious and neglected pathogens, with a focus on biobanking and biosecurity. The consortium has been able to establish it self as a leading voice, drawing attention to scientific infrastructure gaps, the importance of cultural sensitivities, and the power of community engagement. The GET consortium demonstrates how civil society can work together, encourage government engagement and strengthen national and regional efforts to build capacity. PMID:28154625
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Managing crises through organisational development: a conceptual framework.
Lalonde, Carole
2011-04-01
This paper presents a synthesis of the guiding principles in crisis management in accordance with the four configurational imperatives (strategy, structure, leadership and environment) defined by Miller (1987) and outlines interventions in organisational development (OD) that may contribute to their achievement. The aim is to build a conceptual framework at the intersection of these two fields that could help to strengthen the resilient capabilities of individuals, organisations and communities to face crises. This incursion into the field of OD--to generate more efficient configurations of practices in crisis management--seems particularly fruitful considering the system-wide application of OD, based on open-systems theory (Burke, 2008). Various interventions proposed by OD in terms of human processes, structural designs and human resource management, as well as strategy, may help leaders, members of organisations and civil society apply effectively, and in a more sustainable way, the crisis management guiding principles defined by researchers. © 2011 The Author(s). Disasters © Overseas Development Institute, 2011.
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Abayomi, Akin; Gevao, Sahr; Conton, Brian; Deblasio, Pasquale; Katz, Rebecca
2016-01-01
This paper describes the formation of a civil society consortium, spurred to action by frustration over the Ebola crises, to facilitate the development of infrastructure and frameworks including policy development to support a harmonized, African approach to health crises on the continent. The Global Emerging Pathogens Treatment Consortium, or GET, is an important example of how African academics, scientists, clinicians and civil society have come together to initiate policy research, multilevel advocacy and implementation of initiatives aimed at building African capacity for timely and effective mitigations strategies against emerging infectious and neglected pathogens, with a focus on biobanking and biosecurity. The consortium has been able to establish it self as a leading voice, drawing attention to scientific infrastructure gaps, the importance of cultural sensitivities, and the power of community engagement. The GET consortium demonstrates how civil society can work together, encourage government engagement and strengthen national and regional efforts to build capacity.
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Communications Contingency Plan: Planning for Crises and Controversy. Phase 1
NASA Technical Reports Server (NTRS)
Treise, Deborah; Bernstein, Arla G.; Yates, Brad
1998-01-01
Interviews were conducted with a variety of Marshall Space Flight Center personnel and local media representatives in Huntsville, Alabama, in order to identify the current perceptions of these individuals regarding communication effectiveness between MSFC and the media. The purposes of the Phase One report are to (1) assess the need for a contingency plan for communicating in situations of crisis and controversy; (2) identify goals and objectives for the planning process; and (3) provide recommendations for future planning activities to achieve the goals and objectives outlined in Phase One. It is strongly recommended that MSFC personnel who are involved in communications with the media participate in a facilitated, strategic communications planning process in order to develop Phase Two of the Communications Contingency Plan (CCP). Phase Two will address (1) the categorizing, ranking and prioritizing of crises and controversies; (2) the development of action steps and implementation strategies for the CCP; and (3) the development of a monitoring and evaluation process for ongoing plan effectiveness.
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Pawelec, Katarzyna; Salamonowicz, Malgorzata; Panasiuk, Anna; Matysiak, Michal; Demkow, Urszula
2013-01-01
In the present study we investigated the occurrence of systemic and respiratory infections in a cohort of 123 children with severe acquired aplastic anemia (SAA) on immunosuppressive therapy (IST). We recorded 101 episodes of infection in 77 patients (62.6 %). Pneumonia was among the most frequently observed clinical forms of infection (17 cases - 16.8 %). In the entire group, 23 children died, mostly in the course of fatal sepsis (15/23) and in 3 cases because of pneumonia complications. All patients were treated with horse (h-ATG) or rabbit antithymocyte globulin (r-ATG) supplemented with cyclosporine and corticosteroids. The crude incidence rate for serious infections in h-ATG group and r-ATG group was comparable. The relative risk of infectious complications was lower in patients treated with granulocyte colony stimulating factors (G-CSF) by 36 % (RR 0.64; p < 0.0001). The analysis confirmed that respiratory tract and disseminated infections comprise a very serious clinical problem and are the leading cause of death of SAA children. Active surveillance and the analysis of associated risk factors are required to detect opportunistic infections in this group of patients.
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Canalejo, K; Galassi, N; Riera, N; Bengió, R; Aixalá, M
2001-01-01
The expansion of paroxysmal nocturnal hemoglobinuria (PHN) clone was evaluated in a patient with aplastic anemia (AA) of 18 years of evolution during an hemolytic crisis. On day 0, Ham and Sucrosa tests were positive and hematological parameters were altered. Low hemoglobin (Hb) levels and erythrocyte and leukocyte counts were found and continued decreasing on days 7 and 24 (last day of study). High LDH levels, indirect bilirubin and reticulocyte counts were detected throughout. We evaluated CD55 and CD59 on erythrocytes by flow cytometry. Our results showed low CD55 expression with respect to the normal pattern. Since day 0, CD59 staining detected two red cell populations: PNH I (48%), cells with positive fluorescence similar to normal and PNH III (52%), negative cells (PNH clone). These negative cells increased, reaching 70% on day 24. Other membrane anchored leukocyte proteins were also absent (CD14) or decreased (CD16). We found a good correlation between clinical observations, evolution of the laboratory values and expansion of the PNH clone.
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Sarashina, Takeo; Yoshida, Makoto; Iguchi, Akihiro; Okubo, Hitoshi; Toriumi, Naohisa; Suzuki, Daisuke; Sano, Hirozumi; Kobayashi, Ryoji
2013-01-01
Bloodstream infection (BSI) is a recognized cause of morbidity and mortality in children after hematopoietic stem cell transplantation (HSCT). However, there are limited reports on BSI after HSCT in pediatric patients in multiple centers. This study was a retrospective cohort analysis of consecutive patients who underwent allogeneic and autologous HSCT at the Department of Paediatrics, Hokkaido University Hospital, between 1988 and 2009; the Department of Paediatrics, Sapporo Hokuyu Hospital, between 2007 and 2009; and the Department of Paediatrics, Asahikawa Medical University, between 1989 and 2009. A total of 277 patients underwent HSCT during the study period. In this multicenter analysis, cases of BSI after HSCT were recorded in the early posttransplant period (within the first 100 d), and BSI was observed in 24 of 277 HSCT patients. Multivariate analysis showed that nonmalignant disease was an independent factor associated with BSI after HSCT (hazard ratio 6.3 for aplastic anemia or Wiskott-Aldrich syndrome patients; confidence interval, 1.4-12.8; P = 0.012). We conclude that aplastic anemia and Wiskott-Aldrich syndrome were the novel risk factors for BSI in pediatric patients after HSCT.
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[Correlations between asthmatic crisis and meteorological conditions in Rosario, Argentina].
Arnolt, R G; Hoffmann, J A; Daguerre, N; Calcagno, L J
1984-01-01
The aim of this work was to find out whether there is any relationship between the climate in Rosario City, Argentina, and the frequency of asthmatic crises, and whether it would be possible to use weather forecasts to support therapeutic measures. Synoptic and statistical metereological analyses, were used, and the following results achieved: The 721 crises of asthma developed in Rosario City between August 1, 1978 and July 31, 1979, demonstrated the well-known seasonal distribution of similar biological events: high frequencies in the Spring and Fall and low ones in the Summer and Winter. Important correlations were found with high altitude depression systems Hence, in the great majority of cases, the daily frequency of crises attained its maximum values when a depression system in the 500 mb. level was approximately between 60 degrees and 72 degrees W of G. and returned to minimum values when the mentioned system, in its general displacement towards the east, passed the meridian of Rosario City, whose geographic coordinates are: Lat. 32 degrees 55' S, Long. 60 degrees 47' W of G., at 27 meters above sea level. In short, we can say that, as occurs in other parts of the world, the asthmatic crises undergone by children in Rosario City are produced with much more frequency in circumstances of cyclonic atmospheric circulation. Although these situations, in the case of Rosario City, are determined by the dynamic processes in the higher levels of the troposphere. This is probably due to the role played by the Andres Mountain Range in the processes of atmospheric circulation in subtropical South America.(ABSTRACT TRUNCATED AT 250 WORDS)
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Gaponova, N I; Plavunov, N F; Tereshchenko, S N; Baratashvili, V L; Abdurakhmanov, V R; Komissarenko, I A; Filippov, D V; Podkopaev, D V
2011-01-01
Clinicostatistical analysis of arterial hypertension complicated with hypertensive crisis using data of Moscow A.S.Puchkov Station of Urgent and Emergent Medical Aid revealed 14% rise in number of hypertensive crises during the period from 2005 to 2009. Number of hypertensive crises increased among persons of young age (18-35 years). Frequency of cerebrovascular complications of hypertensive crises was age dependent with maximal values among men aged 36-74 years and women older than 75 years.
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Brinkman, Henk-Jan; de Pee, Saskia; Sanogo, Issa; Subran, Ludovic; Bloem, Martin W
2010-01-01
A global economic and financial crisis is engulfing the developing world, coming on top of high food and fuel prices. This paper assesses the impact of the crises on food consumption, nutrition, and health. Several methods were applied, including risk analysis using the cost of the food basket, assessment surveys, simulations, regression analysis using a food consumption score (FCS), reflecting diet frequency and diversity, and a review of the impact of such dietary changes on nutritional status and health. The cost of the food basket increased in several countries, forcing households to reduce quality and quantity of food consumed. The FCS, which is a measure of diet diversity, is negatively correlated with food prices. Simulations show that energy consumption declined during 2006-2010 in nearly all developing regions, resulting potentially in an additional 457 million people (of 4.5 billion) at risk of being hungry and many more unable to afford the dietary quality required to perform, develop, and grow well. As a result of the crises, large numbers of vulnerable households have reduced the quality and quantity of foods they consume and are at risk of increased malnutrition. Population groups most affected are those with the highest requirements, including young children, pregnant and lactating women, and the chronically ill (particularly people with HIV/AIDS and tuberculosis). Because undernutrition during the first 2 y of life has life-long consequences, even short-term price rises will have long-term effects. Thus, measures to mitigate the impact of the crises are urgently required.
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Lehto, Juhani; Vrangbæk, Karsten; Winblad, Ulrika
2015-01-01
Denmark, Finland and Sweden have experienced two major recessions during the last 25 years. The adjustments to the earlier crisis in the late 1980s (Denmark) and early 1990s (Finland and Sweden) resembled the policies in many other European countries during the present crisis. The analysis of relationship of deep economic crises and growth period between them to the health system policies and institutions in the three countries from the 1980s to 2013 is based on a categorisation of reactions to external shocks as path conforming or path breaking. The results of the empirical long-term trends show that the reactions to deep recessions have been mainly temporary adjustments and acceleration of changes already prepared before economic crisis. The economic crisis in the three countries has not been 'good enough' to enable paradigmatic changes in the Nordic public, decentralised and equity-oriented health systems. Changes such as the slow privatisation in care funding and production and the adoption of new management practices indicate an ongoing paradigmatic change related to longer-term societal, ideological and political developments rather than directly to economic crises or growth.
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Quick response airborne command post communications
NASA Astrophysics Data System (ADS)
Blaisdell, Randy L.
1988-08-01
National emergencies and strategic crises come in all forms and sizes ranging from natural disasters at one end of the scale up to and including global nuclear warfare at the other. Since the early 1960s the U.S. Government has spent billions of dollars fielding airborne command posts to ensure continuity of government and the command and control function during times of theater conventional, theater nuclear, and global nuclear warfare. Unfortunately, cost has prevented the extension of the airborne command post technology developed for these relatively unlikely events to the lower level, though much more likely to occur, crises such as natural disasters, terrorist acts, political insurgencies, etc. This thesis proposes the implementation of an economical airborne command post concept to address the wide variety of crises ignored by existing military airborne command posts. The system is known as the Quick Response Airborne Command Post (QRAC Post) and is based on the exclusive use of commercially owned and operated aircraft, and commercially available automated data processing and communications resources. The thesis addresses the QRAC Post concept at a systems level and is primarily intended to demonstrate how current technology can be exploited to economically achieve a national objective.
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Dufour, Carlo; Veys, Paul; Carraro, Elisa; Bhatnagar, Neha; Pillon, Marta; Wynn, Rob; Gibson, Brenda; Vora, Ajay J; Steward, Colin G; Ewins, Anna M; Hough, Rachael E; de la Fuente, Josu; Velangi, Mark; Amrolia, Persis J; Skinner, Roderick; Bacigalupo, Andrea; Risitano, Antonio M; Socie, Gerard; Peffault de Latour, Regis; Passweg, Jakob; Rovo, Alicia; Tichelli, André; Schrezenmeier, Hubert; Hochsmann, Britta; Bader, Peter; van Biezen, Anja; Aljurf, Mahmoud D; Kulasekararaj, Austin; Marsh, Judith C; Samarasinghe, Sujith
2015-11-01
We explored the feasibility of unrelated donor haematopoietic stem cell transplant (HSCT) upfront without prior immunosuppressive therapy (IST) in paediatric idiopathic severe aplastic anaemia (SAA). This cohort was then compared to matched historical controls who had undergone first-line therapy with a matched sibling/family donor (MSD) HSCT (n = 87) or IST with horse antithymocyte globulin and ciclosporin (n = 58) or second-line therapy with unrelated donor HSCT post-failed IST (n = 24). The 2-year overall survival in the upfront cohort was 96 ± 4% compared to 91 ± 3% in the MSD controls (P = 0·30) and 94 ± 3% in the IST controls (P = 0·68) and 74 ± 9% in the unrelated donor HSCT post-IST failure controls (P = 0·02).The 2-year event-free survival in the upfront cohort was 92 ± 5% compared to 87 ± 4% in MSD controls (P = 0·37), 40 ± 7% in IST controls (P = 0·0001) and 74 ± 9% in the unrelated donor HSCT post-IST failure controls (n = 24) (P = 0·02). Outcomes for upfront-unrelated donor HSCT in paediatric idiopathic SAA were similar to MSD HSCT and superior to IST and unrelated donor HSCT post-IST failure. Front-line therapy with matched unrelated donor HSCT is a novel treatment approach and could be considered as first-line therapy in selected paediatric patients who lack a MSD. © 2015 John Wiley & Sons Ltd.
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[Cardiovascular complications of hypertensive crisis].
Rosas-Peralta, Martín; Borrayo-Sánchez, Gabriela; Madrid-Miller, Alejandra; Ramírez-Arias, Erick; Pérez-Rodríguez, Gilberto
2016-01-01
It is inexorable that a proportion of patients with systemic arterial hypertension will develop a hypertensive crisis at some point in their lives. The hypertensive crises can be divided in hypertensive patients with emergency or hypertensive emergency, according to the presence or absence of acute end-organ damage. In this review, we discuss the cardiovascular hypertensive emergencies, including acute coronary syndrome, congestive heart failure, aortic dissection and sympathomimetic hypertensive crises (those caused by cocaine use included). Each is presented in a unique way, although some patients with hypertensive emergency report non-specific symptoms. Treatment includes multiple medications for quick and effective action with security to reduce blood pressure, protect the function of organs remaining, relieve symptoms, minimize the risk of complications and improve patient outcomes.
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Estrogen-induced myelotoxicity in dogs: A review
Sontas, Hasan B.; Dokuzeylu, Banu; Turna, Ozge; Ekici, Hayri
2009-01-01
Exogenous estrogens used for therapeutic purposes or endogenous estrogen sources such as functional Sertoli cell or ovarian granulosa cell tumors may cause bone marrow toxicity in dogs. The condition is characterized by hematologic abnormalities including thrombocytopenia, anemia, and leukocytosis or leukopenia. Despite intensive therapy with blood or platelet-rich transfusions, broad-spectrum antibiotics, steroids, and bone marrow stimulants, prognosis is unfavorable. Due to the the risk of stimulating the development of uterine diseases and the potential for inducing aplastic anemia, estrogen use in dogs is best avoided where possible. This paper describes the causes of estrogen-induced myelotoxicity, the clinical presentation of the patients, the diagnosis, and the treatment options in the dog. PMID:20046604
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Health workforce and governance: the crisis in Nigeria.
Adeloye, Davies; David, Rotimi Adedeji; Olaogun, Adenike Ayobola; Auta, Asa; Adesokan, Adedapo; Gadanya, Muktar; Opele, Jacob Kehinde; Owagbemi, Oluwafemi; Iseolorunkanmi, Alexander
2017-05-12
In Nigeria, several challenges have been reported within the health sector, especially in training, funding, employment, and deployment of the health workforce. We aimed to review recent health workforce crises in the Nigerian health sector to identify key underlying causes and provide recommendations toward preventing and/or managing potential future crises in Nigeria. We conducted a scoping literature search of PubMed to identify studies on health workforce and health governance in Nigeria. A critical analysis, with extended commentary, on recent health workforce crises (2010-2016) and the health system in Nigeria was conducted. The Nigerian health system is relatively weak, and there is yet a coordinated response across the country. A number of health workforce crises have been reported in recent times due to several months' salaries owed, poor welfare, lack of appropriate health facilities and emerging factions among health workers. Poor administration and response across different levels of government have played contributory roles to further internal crises among health workers, with different factions engaged in protracted supremacy challenge. These crises have consequently prevented optimal healthcare delivery to the Nigerian population. An encompassing stakeholders' forum in the Nigerian health sector remain essential. The national health system needs a solid administrative policy foundation that allows coordination of priorities and partnerships in the health workforce and among various stakeholders. It is hoped that this paper may prompt relevant reforms in health workforce and governance in Nigeria toward better health service delivery in the country.
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ERIC Educational Resources Information Center
DeFigio, Nicholas; Hughes, Sean
Increasingly, principals are being viewed as instructional leaders, professional educators active in initiating and planning teacher development programs. While most principals consider instructional leadership a high priority, they spend much of their time solving routine problems and confronting minor crises. Immediate, short-term responses take…
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Psychosocial Development and the Big Five Personality Traits among Chinese University Students
ERIC Educational Resources Information Center
Zhang, Li-fang
2013-01-01
This study explores how psychosocial development and personality traits are related. In particular, the study investigates the predictive power of the successful resolution of the Eriksonian psychosocial crises for the Big Five personality traits beyond age and gender. Four hundred university students in mainland China responded to the Measures of…
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Exploring the Strategic Role of Human Resource Development in Organizational Crisis Management
ERIC Educational Resources Information Center
Wang, Jia; Hutchins, Holly M.; Garavan, Thomas N.
2009-01-01
Crisis management has been a largely overlooked territory in human resource development (HRD) despite the increasingly recognized impact of organizational crises on the individual and organizational performance. This article explores the strategic role of HRD in the context of organizational crisis management using Garavan's strategic HRD model as…
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Kumasaka, Y.
The author analyzed the slowdown in productivity growth since the first oil crisis for about ten industrial sectors in both Japan and the USA and also compared the similarities and differences of their economic performances from the two oil crises. It is assumed that the increase in energy prices was the main cause of the slowdown in productivity growth. The productivity experiences from the two oil crises through the shift of factor price frontier, which was obtained from the cost function, was analyzed. The two oil crises had much responsibility for the slowdown in productivity growth since 1973. However, themore » effects of the oil crises on production were quite different not only among industrial sectors but also between Japan and the USA. The mining sector suffered from the two oil crises most severely in both countries. The Japanese transportation and communication sector and the US communication sector suffered from them least severely. Also studied was the learning effect from the first oil crisis for the Japanese manufacturing and the US commercial sectors. The reason why most of Japanese industrial sectors managed the second oil crisis relatively well was due to the downward flexibility of their real wages during the second oil crisis.« less
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Chen, Thai-Form; Tseng, Hsing-Chau
2006-02-01
Extensive media coverage is warning of a crisis of trust that has emerged as a serious issue in our society. This article explores the meaning of "crisis," concepts of crisis management, mechanisms for building trust, and the underlying significance of trust and distrust. Evidence is adduced to testify to the erosion of trust and factors in our society that reflect the potential for crises of trust. Organizational decision makers, including hospital managers, are urged to identify and reflect upon weaknesses in their organizations in order that remedial action can be taken to preempt such crises.
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1999-11-01
C17 C130 + $2B upgrade SSTOL VTOL Advanced VTOL 6060120150 Vehicle wt. (tons) Ktons 2015 26 U.S. military dominance in future crises . It is also a...improvement between crises . A “pick-up” approach to C4ISR systems is not effective for today’s complex contingencies, yet it has become the norm. Instead...demands during crises and to stress imposed on the system by adversaries. The infrastructure must allow information to be distributed to and from any
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ERIC Educational Resources Information Center
Molla, Tsegaye
2017-01-01
The difference in economic development among nations entirely emanates from difference in human capital development as it is the priority pathway out of poverty, diverse socio-economic and environmental crises. Although, huge investment in human capital development has long been made, mere investment will never lead to quality labor force unless…
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Identity Transformation of Korean Immigrants.
ERIC Educational Resources Information Center
Kim, Saekyung; Gaa, John; Swank, Paul; Liberman, Dov
Immigration is one of the most significant changes which can occur in one's lifetime. Immigrants struggle with their foreign environment and renewed crises; they suffer from "uprootedness" and "missed embeddedness" and have difficulty integrating their identity roles. Erikson's psychosocial development theory and Marcia's…
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Adjustment, social sectors, and demographic change in Sub-Saharan Africa.
Ekouevi, K; Adepoju, A
1995-01-01
This discussion concludes that the economic crises of the 1980s resulted in a halt to the social and economic development of sub-Saharan Africa. Employment, health, and education sectors all deteriorated under structural adjustment programs (SAPs) and poor economic performance. SAPs are considered inadequate solutions to long-term problems. Economic crises were found to affect countries differently in their demographic impact. Delayed demographic transition occurred both through economic development as a prerequisite and as a result of poor economic development. Case studies of each country are considered the appropriate geographic unit of analysis of demographic change rather than regional or comparative studies. The economic crises in sub-Saharan Africa occurred due to both external (commodity prices, high real interest rates, and decreasing net capital flows) and internal distortions (strategies of development such as import substitution, neglect of the agricultural sector, and government control of prices and trade). The unfavorable external context reduced export prices and earnings while increasing the costs of imports. Internal controls were detrimental to farmers. During the 1970s and 1980s African countries experienced declines in both the volume and value of exports, increases in import volume, and imbalances in the balance of payments. Large domestic borrowing and foreign borrowing was done by governments, which was at the expense of the private sector. Economic management and corruption were rampant. SAPs restrained demand, reduced public expenditures, adjusted exchange rates, contracted the size of the public sector, liberalized trade, deregulated the interest rate, stimulated domestic production, and used market forces for balancing optimum allocation of resources. SAPs were the fix for trade imbalances and government debt. Development was slowed or stopped. During 1980-87 spending on health care, education, and infrastructure was drastically reduced. These already weak sectors were further weakened. Inflation rose. Public sector employment was reduced. Wages declined, which resulted in a massive demoralization, unemployment, and poverty. Manpower development was threatened by declines in education.
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Ashraf, Asma; Hadjinicolaou, Andreas V; Doree, Carolyn; Hopewell, Sally; Trivella, Marialena; Estcourt, Lise J
2016-01-01
This is the protocol for a review and there is no abstract. The objectives are as follows: To compare a therapeutic-only versus prophylactic platelet transfusion policy for people with myelodysplasia, inherited or acquired aplastic anaemia, and other congenital bone marrow failure disorders. PMID:27660553
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Understanding and Targeting Epigenetic Alterations in Acquired Bone Marrow Failure
2014-05-01
2013 Dept. of Cell and Molecular Biology, Chiba University, Chiba Japan 2013 ASH Educational Session on Myeloproliferative Neoplasms 2014 Aplastic... myeloproliferative neoplasms (MPNs). These CORRESPONDENCE Ross L. Levine: leviner@mskcc.org OR Iannis Aifantis: iannis.aifantis@nyumc.org...myeloid progenitor; MPN, myeloproliferative neoplasm ; MPP, multipotent progenitor; polyI:polyC, polyinosinic- polycytidylic acid; qRT-PCR
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Dynamic changes of striatal and extrastriatal abnormalities in glutaric aciduria type I.
Harting, Inga; Neumaier-Probst, Eva; Seitz, Angelika; Maier, Esther M; Assmann, Birgit; Baric, Ivo; Troncoso, Monica; Mühlhausen, Chris; Zschocke, Johannes; Boy, Nikolas P S; Hoffmann, Georg F; Garbade, Sven F; Kölker, Stefan
2009-07-01
In glutaric aciduria type I, an autosomal recessive disease of mitochondrial lysine, hydroxylysine and tryptophan catabolism, striatal lesions are characteristically induced by acute encephalopathic crises during a finite period of brain development (age 3-36 months). The frequency of striatal injury is significantly less in patients diagnosed as asymptomatic newborns by newborn screening. Most previous studies have focused on the onset and mechanism of striatal injury, whereas little is known about neuroradiological abnormalities in pre-symptomatically diagnosed patients and about dynamic changes of extrastriatal abnormalities. Thus, the major aim of the present retrospective study was to improve our understanding of striatal and extrastriatal abnormalities in affected individuals including those diagnosed by newborn screening. To this end, we systematically analysed magnetic resonance imagings (MRIs) in 38 patients with glutaric aciduria type I diagnosed before or after the manifestation of neurological symptoms. To identify brain regions that are susceptible to cerebral injury during acute encephalopathic crises, we compared the frequency of magnetic resonance abnormalities in patients with and without such crises. Major specific changes after encephalopathic crises were found in the putamen (P < 0.001), nucleus caudatus (P < 0.001), globus pallidus (P = 0.012) and ventricles (P = 0.001). Analysis of empirical cumulative distribution frequencies, however, demonstrated that isolated pallidal abnormalities did not significantly differ over time in both groups (P = 0.544) suggesting that isolated pallidal abnormalities are not induced by acute crises--in contrast to striatal abnormalities. The manifestation of motor disability was associated with signal abnormalities in putamen, caudate, pallidum and ventricles. In addition, we found a large number of extrastriatal abnormalities in patients with and without preceding encephalophatic crises. These abnormalities include widening of anterior temporal and sylvian CSF spaces, pseudocysts, signal changes of substantia nigra, nucleus dentatus, thalamus, tractus tegmentalis centralis and supratentorial white matter as well as signs of delayed maturation (myelination and gyral pattern). In contrast to the striatum, extrastriatal abnormalities were variable and could regress or even normalize with time. This includes widening of sylvian fissures, delayed maturation, pallidal signal changes and pseudocysts. Based on these results, we hypothesize that neuroradiological abnormalities and neurological symptoms in glutaric aciduria type I can be explained by overlaying episodes of cerebral alterations including maturational delay of the brain in utero, acute striatal injury during a vulnerable period in infancy and chronic progressive changes that may continue lifelong. This may have widespread consequences for the pathophysiological understanding of this disease, long-term outcomes and therapeutic considerations.
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de Goeij, Moniek C M; Suhrcke, Marc; Toffolutti, Veronica; van de Mheen, Dike; Schoenmakers, Tim M; Kunst, Anton E
2015-04-01
Economic crises are complex events that affect behavioral patterns (including alcohol consumption) via opposing mechanisms. With this realist systematic review, we aimed to investigate evidence from studies of previous or ongoing crises on which mechanisms (How?) play a role among which individuals (Whom?). Such evidence would help understand and predict the potential impact of economic crises on alcohol consumption. Medical, psychological, social, and economic databases were used to search for peer-reviewed qualitative or quantitative empirical evidence (published January 1, 1990-May 1, 2014) linking economic crises or stressors with alcohol consumption and alcohol-related health problems. We included 35 papers, based on defined selection criteria. From these papers, we extracted evidence on mechanism(s), determinant, outcome, country-level context, and individual context. We found 16 studies that reported evidence completely covering two behavioral mechanisms by which economic crises can influence alcohol consumption and alcohol-related health problems. The first mechanism suggests that psychological distress triggered by unemployment and income reductions can increase drinking problems. The second mechanism suggests that due to tighter budget constraints, less money is spent on alcoholic beverages. Across many countries, the psychological distress mechanism was observed mainly in men. The tighter budget constraints mechanism seems to play a role in all population subgroups across all countries. For the other three mechanisms (i.e., deterioration in the social situation, fear of losing one's job, and increased non-working time), empirical evidence was scarce or absent, or had small to moderate coverage. This was also the case for important influential contextual factors described in our initial theoretical framework. This realist systematic review suggests that among men (but not among women), the net impact of economic crises will be an increase in harmful drinking. Such a different net impact between men and women could potentially contribute to growing gender-related health inequalities during a crisis. Copyright © 2015 Elsevier Ltd. All rights reserved.
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Fottrell, E; Byass, P
2009-02-01
Effective early warning systems of humanitarian crises may help to avert substantial increases in mortality and morbidity, and prevent major population movements. The Butajira Rural Health Programme (BRHP) in Ethiopia has maintained a programme of epidemiological surveillance since 1987. Inspection of the BRHP data revealed large peaks of mortality in 1998 and 1999, well in excess of the normally observed year-to-year variation. Further investigation and enquiry revealed that these peaks related to a measles epidemic, and a serious episode of drought and consequent food insecurity that went undetected by the BRHP. This paper applies international humanitarian crisis threshold definitions to the BRHP data in an attempt to identify suitable mortality thresholds that may be used for the prospective detection of humanitarian crises in population surveillance sites in developing countries. Empirical investigation using secondary analysis of longitudinal population-based cohort data. The daily, weekly and monthly thresholds for crises in Butajira were applied to mortality data for the 5-year period incorporating the crisis periods of 1998-1999. Days, weeks and months in which mortality exceeded each threshold level were identified. Each threshold level was assessed in terms of prospectively identifying the true crisis periods in a timely manner whilst avoiding false alarms. The daily threshold definition is too sensitive to accurately detect impending or real crises in the population surveillance setting of the BRHP. However, the weekly threshold level is useful in identifying important increases in mortality in a timely manner without the excessive sensitivity of the daily threshold. The weekly threshold level detects the crisis periods approximately 2 weeks before the monthly threshold level. Mortality measures are highly specific indicators of the health status of populations, and simple procedures can be used to apply international crisis threshold definitions in population surveillance settings for the prospective detection of important changes in mortality rate. Standards for the timely use of surveillance data and ethical responsibilities of those responsible for the data should be made explicit to improve the public health functioning of current sentinel surveillance methodologies.
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Deferred School Maintenance Creates National Crises.
ERIC Educational Resources Information Center
Geiger, Philip E.
2002-01-01
Describes the cost and causes of the school maintenance "crises"; lists seven questions to determine if a school district has a quality maintenance program; describes consequences of deferred school maintenance in Yuma (Arizona) Union High School District. (PKP)
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The benefits of flexible team interaction during crises.
Stachowski, Alicia A; Kaplan, Seth A; Waller, Mary J
2009-11-01
Organizations increasingly rely on teams to respond to crises. While research on team effectiveness during nonroutine events is growing, naturalistic studies examining team behaviors during crises are relatively scarce. Furthermore, the relevant literature offers competing theoretical rationales concerning effective team response to crises. In this article, the authors investigate whether high- versus average-performing teams can be distinguished on the basis of the number and complexity of their interaction patterns. Using behavioral observation methodology, the authors coded the discrete verbal and nonverbal behaviors of 14 nuclear power plant control room crews as they responded to a simulated crisis. Pattern detection software revealed systematic differences among crews in their patterns of interaction. Mean comparisons and discriminant function analysis indicated that higher performing crews exhibited fewer, shorter, and less complex interaction patterns. These results illustrate the limitations of standardized response patterns and highlight the importance of team adaptability. Implications for future research and for team training are included.
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Exploring Crisis Management in U.S. Small Businesses
NASA Astrophysics Data System (ADS)
Williams, Jon
As a critical infrastructure, the US electricity grid supplies electricity to 340 million people within eight separate regions. The power infrastructure is vulnerable to many types of disasters capable of severing supplies of electricity. The impact on the employees and communities when small- and medium-size enterprises are shut down due to disasters can be severe. The purpose of the quantitative comparative study was to explore small- and medium-size enterprises crisis management strategies in the case of power infrastructure vulnerabilities. Perceptions of small business leaders were probed about crisis management planning relevant to three secondary factors: prior experience of crises, threat perceptions, and planning self-efficacy. Participants completed an adapted questionnaire instrument based on a five-point Likert scale for six sub-factors including resilience through planning, financial impact, operational crisis management, the perfect storm, the aftermath of survival, and atrophy. The instrument also measured three additional factors to include, prior experience of crises, threat perceptions, and planning self-efficacy, across seven types of crises. The results of this study indicated that of the 276 respondents, 104 had no crisis plans, but 172 did have crisis plans. Of those who had implemented crisis plans, 19% had specific provisions to address power outages or attacks on the electrical grid. Of the respondents who had not planned for power outages nor experienced significant losses of power, a statistically significant number acknowledged an external threat to their business. The majority of respondents indicated that long-term planning was related to resilience; however, the migration of crisis understanding into the planning process or implementation was not implemented. This heightened awareness of potential crises without the corresponding development and implementation of mitigation crisis plans requires additional research to understand drivers effecting the decision making process with crisis managers.
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NASA Astrophysics Data System (ADS)
Uritskaya, Olga Y.
2005-05-01
Results of fractal stability analysis of daily exchange rate fluctuations of more than 30 floating currencies for a 10-year period are presented. It is shown for the first time that small- and large-scale dynamical instabilities of national monetary systems correlate with deviations of the detrended fluctuation analysis (DFA) exponent from the value 1.5 predicted by the efficient market hypothesis. The observed dependence is used for classification of long-term stability of floating exchange rates as well as for revealing various forms of distortion of stable currency dynamics prior to large-scale crises. A normal range of DFA exponents consistent with crisis-free long-term exchange rate fluctuations is determined, and several typical scenarios of unstable currency dynamics with DFA exponents fluctuating beyond the normal range are identified. It is shown that monetary crashes are usually preceded by prolonged periods of abnormal (decreased or increased) DFA exponent, with the after-crash exponent tending to the value 1.5 indicating a more reliable exchange rate dynamics. Statistically significant regression relations (R=0.99, p<0.01) between duration and magnitude of currency crises and the degree of distortion of monofractal patterns of exchange rate dynamics are found. It is demonstrated that the parameters of these relations characterizing small- and large-scale crises are nearly equal, which implies a common instability mechanism underlying these events. The obtained dependences have been used as a basic ingredient of a forecasting technique which provided correct in-sample predictions of monetary crisis magnitude and duration over various time scales. The developed technique can be recommended for real-time monitoring of dynamical stability of floating exchange rate systems and creating advanced early-warning-system models for currency crisis prevention.
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A Systematic Review on Health Resilience to Economic Crises
Glonti, Ketevan; Gordeev, Vladimir S.; Goryakin, Yevgeniy; Reeves, Aaron; Stuckler, David; McKee, Martin; Roberts, Bayard
2015-01-01
Background The health effects of recent economic crises differ markedly by population group. The objective of this systematic review is to examine evidence from longitudinal studies on factors influencing resilience for any health outcome or health behaviour among the general population living in countries exposed to financial crises. Methods We systematically reviewed studies from six electronic databases (EMBASE, Global Health, MEDLINE, PsycINFO, Scopus, Web of Science) which used quantitative longitudinal study designs and included: (i) exposure to an economic crisis; (ii) changes in health outcomes/behaviours over time; (iii) statistical tests of associations of health risk and/or protective factors with health outcomes/behaviours. The quality of the selected studies was appraised using the Quality Assessment Tool for Quantitative Studies. PRISMA reporting guidelines were followed. Results From 14,584 retrieved records, 22 studies met the eligibility criteria. These studies were conducted across 10 countries in Asia, Europe and North America over the past two decades. Ten socio-demographic factors that increased or protected against health risk were identified: gender, age, education, marital status, household size, employment/occupation, income/ financial constraints, personal beliefs, health status, area of residence, and social relations. These studies addressed physical health, mortality, suicide and suicide attempts, mental health, and health behaviours. Women’s mental health appeared more susceptible to crises than men’s. Lower income levels were associated with greater increases in cardiovascular disease, mortality and worse mental health. Employment status was associated with changes in mental health. Associations with age, marital status, and education were less consistent, although higher education was associated with healthier behaviours. Conclusions Despite widespread rhetoric about the importance of resilience, there was a dearth of studies which operationalised resilience factors. Future conceptual and empirical research is needed to develop the epidemiology of resilience. PMID:25905629
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State network approach to characteristics of financial crises
NASA Astrophysics Data System (ADS)
Qiu, Lu; Gu, Changgui; Xiao, Qin; Yang, Huijie; Wu, Guolin
2018-02-01
Extensive works have reported that a financial crisis can induce significant changes to topological structure of a stock network constructed with cross-correlations between stocks. But there are still some problems to be answered, such as what is the relationship between different crises in history and how to classify them? In the present work, we propose a new network-based solution to extract and display the relationships between the crises. The Dow Jones stock market is investigated as a typical example. The cross-correlation matrix between stocks is used to measure the state of stock market, called state matrix. All the states cluster into six sub-categories. A state network is constructed further to display the relationships between all the states, which contains a total of nine communities. It is found that three crises C , D and E (refer to the Lehman's bankruptcy in 2008, the Euro-zone and International Monetary Fund decide the first bailout for Greece in 2010, and the European sovereign debt crisis in 2011, respectively) belong to a specific sub-category and cluster in a single community. The mid-stage of C is closely linked with E, while the other stages with D. The other two crises A and B (refer to the financial crisis in Asia in 1997, and the burst of "dot-com bubble" in 2002, respectively) belong to another sub-category and gather in a corner of another single community. A and B are linked directly with C and D by two edges. By this way, we give a clear picture of the relationships between the crises.
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ERIC Educational Resources Information Center
Perea, Rosalie D.; Morrison, Shirley
1997-01-01
To handle unforeseen crises, Albuquerque Public Schools established a critical-incident response team with a simple, understandable chain of command. The group aims to ensure maximum safety and people' well-being, develop a districtwide crisis-response-management plan, coordinate necessary training, and collaborate with community agencies…
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Crisis Communication Plans: Poor Predictors of Excellent Crisis Public Relations.
ERIC Educational Resources Information Center
Marra, Francis J.
1998-01-01
Argues that newly developed theory in crisis public relations suggests a shift is necessary in the way practitioners view crises. Notes that the new paradigm defines excellent crisis public relations very differently from the literature of the past 20 years. (RS)
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ERIC Educational Resources Information Center
Lelle, Mark A.; Holt, Barbara A.
1987-01-01
The authors focus on providing opportunities for women in Third World countries in agriculture. A review of the body of knowledge in agricultural development and of the issues surrounding current world food crises is included. (CH)
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Stock network stability in times of crisis
NASA Astrophysics Data System (ADS)
Heiberger, Raphael H.
2014-01-01
Despite many efforts crises on financial markets are in large part still scientific black-boxes. In this paper, we use a winner-take-all approach to construct a longitudinal network of S&P 500 companies and their correlations between 2000 and 2012. A comparison to complex ecosystems is drawn, especially whether the May-Wigner theorem can describe real-world economic phenomena. The results confirm the utility of the May-Wigner theorem as a stability indicator for the US stock market, since its development matches with the two major crises of this period, the dot-com bubble and, particularly, the financial crisis. In those times of financial turmoil, the stock network changes its composition, but unlike ecological systems it tightens and the disassortative structure of prosperous markets transforms into a more centralized topology.
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Teaching and Learning for Sustainable Development: ESD Research in Technology Education
ERIC Educational Resources Information Center
Pavlova, Margarita
2013-01-01
When education for sustainable development (ESD) emerged as part of the educational agenda in the international arena, it was associated with significant shifts in the educational debate about the purpose and nature of education and with the need to respond to crises caused by the modern idea of progress. Scientists from different fields warn…
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The Comprehensive School in Spain: A Review of Its Development Cycle and Crises
ERIC Educational Resources Information Center
Bolívar, Antonio
2015-01-01
The purpose of this study is to describe, analyse and evaluate the successive comprehensive reforms in Spain as a "paradigmatic" example of the emergence, evolution and crisis of the comprehensive school. In the first part, we describe the development of the comprehensive school project (1970-2013), using the image of the life cycle,…
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Understanding the importance of an energy crisis
NASA Astrophysics Data System (ADS)
Mechtenberg, Abigail Reid
Human development and energy, in general, and electrical energy, specifically, co-exist seamlessly in high HDI countries where reliability and availability is greater than 99%. In numerous low HDI countries, there is 2-50% electric grid availability with reliability at or below 50% due to load shedding and faults. In Africa, solar, wind, biomass and hydroelectric energy production are cited to meet growing demand and increase reliability and availability; however, the capital costs are greater than the ability-to-pay for wide scale implementation. Since the 1970s, the United States has continued to argue over the new sustainable energy infrastructure solution(s); thus resulting in no new infrastructure being built for wide scale implementation. Together the world is facing the daunting task of averting an energy crisis in developed countries and facing energy crises in developing countries. This thesis explores the importance of energy crises: from the past, current, and future. The first part entails arguing that the United States is not on a pathway to prevent an energy crisis based on an analysis of 1986 and 2004 niche and status-quo manufacturing of light-duty vehicles. The second part answers the question of what an energy crisis looks like by exploring and investigating current electrical energy crises in Fort Portal, Uganda. This part used both anthropological and physics education empowerment research to co-design and build for various energy crisis situations in hospitals, schools, and businesses all from locally available materials and expertise. Finally, looking into the US light-duty vehicle's future, I design a new hybrid vehicle powertrain (called transition mode hybrid). This third part describes my new patent as a way to avert an energy crisis in the light-duty transportation sector.
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Human Needs: A Literature Review and Cognitive Life Span Model
1992-04-01
stages where crises are met, and, if favorably resolved, result in personality growth. Erikson’s psychosocial theory covers development from birth to...death. He describes eight major tasks or challenges people face as they undergo psychosocial development . Three of the eight stages of development ...the order that Erikson suggested. Several researchers (e.g., Rosenthal, Gurney, & Moore, 1981) have developed inventories that measure Erikson’s stages
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2018-05-10
Acute Myeloid Leukemia; Acute Myeloid Leukemia in Remission; Aplastic Anemia; Chronic Myelomonocytic Leukemia; Hodgkin Lymphoma; Indolent Non-Hodgkin Lymphoma; Malignant Neoplasm; Myelodysplastic Syndrome; Myeloproliferative Neoplasm; Plasma Cell Myeloma; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Ring Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Refractory Cytopenia With Multilineage Dysplasia and Ring Sideroblasts
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[Chronologic analysis of clonal evolution in acquired aplastic anemia and sMDS].
Yoshizato, Tetsuichi
2016-04-01
Acquired aplastic anemia (AA) is a prototype of idiopathic bone marrow failure, which is caused by immune-mediated destruction of hematopoietic progenitors but is also characterized by frequent evolution to clonal myeloid disorders, such as myelodysplastic syndromes or acute myeloid leukemia. However, the chronological behavior of the clonality and its link to myelodysplastic syndrome or acute myeloid leukemia has not been fully explored. To define the clonality and its chronological behavior in AA, we performed targeted sequencing (N=439) in cases with AA. Somatic mutations were detected in 1/3 of our cases. Mutations were most frequently found in DNMT3A, followed by BCOR, PIGA and ASXL1. The prevalence of mutations increased with age. The clone sizes in DNMT3A and ASXL1 were prone to increase, whereas those of BCOR and PIGA were more likely to decrease or remain stable. Mutations in PIGA, BCOR and BCORL1 correlated with a better response to immunosuppressive therapy and more favorable survival. On the other hand, other mutations were associated with worse outcomes. The chronological dynamics of clonality showed marked variability and were not necessarily associated with prognosis.
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Aldehyde Dehydrogenase 2 in Aplastic Anemia, Fanconi Anemia and Hematopoietic Stem Cells
Van Wassenhove, Lauren D.; Mochly-Rosen, Daria; Weinberg, Kenneth I.
2016-01-01
Maintenance of the hematopoietic stem cell (HSC) compartment depends on the ability to metabolize exogenously and endogenously generated toxins, and to repair cellular damage caused by such toxins. Reactive aldehydes have been demonstrated to cause specific genotoxic injury, namely DNA interstrand cross-links. Aldehyde dehydrogenase 2 (ALDH2) is a member of a 19 isoenzyme ALDH family with different substrate specificities, subcellular localization, and patterns of expression. ALDH2 is localized in mitochondria and is essential for the metabolism of acetaldehyde, thereby placing it directly downstream of ethanol metabolism. Deficiency in ALDH2 expression and function are caused by a single nucleotide substitution and resulting amino acid change, called ALDH2*2. This genetic polymorphism affects 35–45% of East Asians (about ~560 million people), and causes the well-known Asian flushing syndrome, which results in disulfiram-like reactions after ethanol consumption. Recently, the ALDH2*2 genotype has been found to be associated with marrow failure, with both an increased risk of sporadic aplastic anemia and more rapid progression of Fanconi Anemia. This review discusses the unexpected interrelationship between aldehydes, ALDH2 and hematopoietic stem cell biology, and in particular its relationship to Fanconi anemia. PMID:27650066
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Gandhi, Manish J; Pendergrass, Thomas W; Cummings, Carrie C; Ihara, Kenji; Blau, C Anthony; Drachman, Jonathan G
2005-10-01
An 11-year-old girl, presenting with fatigue and bruising, was found to be profoundly pancytopenic. Bone marrow exam and clinical evaluation were consistent with aplastic anemia. Family members were studied as potential stem cell donors, revealing that both younger siblings displayed significant thrombocytopenia, whereas both parents had normal blood counts. We evaluated this pedigree to understand the unusually late presentation of congenital amegakaryocytic thrombocytopenia (CAMT). The coding region and the intron/exon junctions of MPL were sequenced from each family member. Vectors representing each of the mutations were constructed and tested for the ability to support growth of Baf3/Mpl(mutant) cells. All three siblings had elevated thrombopoietin levels. Analysis of genomic DNA demonstrated that each parent had mutations/polymorphisms in a single MPL allele and that each child was a compound heterozygote, having inherited both abnormal alleles. The maternal allele encoded a mutation of the donor splice-junction at the exon-3/intron-3 boundary. A mini-gene construct encoding normal vs mutant versions of the intron-3 donor-site demonstrated that physiologic splicing was significantly reduced in the mutant construct. Mutations that incompletely eliminate Mpl expression/function may result in delayed diagnosis of CAMT and confusion with aplastic anemia.
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[Clinical study of TCM-WM on aplastic anemia complicated with hepatitis C].
Liu, Q C; Zheng, B R; Zhang, C L
1995-04-01
The testing kit of second generation for serum anti-HCV was used in 82 cases of aplastic anemia (AA). The results showed that positive rate was 69.4% (43/62) in the patients of AA with transfusion, this was significantly higher than that in the patients of AA without transfusion. There was no difference of anti-HCV antibody positive rate between chronic AA and acute AA (P > 0.05), incidence rate of post-transfusion hepatitis C (PTHC) in AA was 33.9% (21/62), among which the incidence rate in acute and chronic AA were 68.8% (11/16) and 21.7% (10/46) respectively (P < 0.01). The anti-HCV positive patients were divided into two groups: PTHC and non-PTHC, there was no statistical difference of their transfusion volume, hemoglobin, white blood cell between these groups. Response rate of AA was lower in anti-HCV positive patients than that in negative patients (P < 0.05). Acute, icteric PTHC was predominant in patients with AA. The patients with AA complicated with PTHC was liable to bleed and be infected. PTHC has been an important complication in patients with AA. The better response was obtained by TCM-WM therapy in the patients.
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Distribution of elements in individual blood cells in metabolic disorders at the cellular level
NASA Astrophysics Data System (ADS)
Johansson, Erland; Lindh, Ulf
1985-08-01
In comparison with controls neutrophil granulocytes from Rheumatoid arthritis (RA), Infantile Neuronal Ceroid Lipofuscinosis (INCL), Chronic Lymphatic Leukemia (L) and Aplastic Anemia (AA) displayed significant alterations in essential and non-essential elements which might be interpreted as fingerprints of these deseases. The neutrophils from RA patients displayed alterations in the concentrations of iron, calcium, strontium, manganese, zinc and copper. INCL displayed alterations in the concentrations of iron and copper but in the INCL disease the iron concentration was about 2 times higher than in RA. In leukemia, aluminium was observed but not in the controls (< 0.5 μg/ g). The zinc concentration was lowered in leukemia. Aplastic anemia displayed alterations in zirconium, arsenic, molybdenum, iron and zinc. The platelets from RA, INCL, L and AA patients also displayed alterations in the elemental profiles. The platelets from AA patients displayed a unique elemental distribution of arsenic, zirconium and molybdenum. The elemental profiles of the thrombocytes and neutrophils might be used as a complement in the diagnosis of the examined diseases and in therapy the elemental profile might be used to monitor drugs at the cellular level.
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DOT National Transportation Integrated Search
2010-07-01
The objective of this work was to develop a : low-cost portable damage detection tool to : assess and predict damage areas in highway : bridges. : The proposed tool was based on standard : vibration-based damage identification (VBDI) : techniques but...
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Nurturing Young Student Mathematicians
ERIC Educational Resources Information Center
Gavin, M. Katherine; Casa, Tutita M.
2013-01-01
Developing mathematical talent in our students should be of primary consideration in education today as nations respond to the challenges of economic crises and ever-changing technological advances. This paper describes two U.S. federally funded curriculum projects, Project M[superscript 3], Mentoring Mathematical Minds, and Project M[superscript…
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Entropies of negative incomes, Pareto-distributed loss, and financial crises.
Gao, Jianbo; Hu, Jing; Mao, Xiang; Zhou, Mi; Gurbaxani, Brian; Lin, Johnny
2011-01-01
Health monitoring of world economy is an important issue, especially in a time of profound economic difficulty world-wide. The most important aspect of health monitoring is to accurately predict economic downturns. To gain insights into how economic crises develop, we present two metrics, positive and negative income entropy and distribution analysis, to analyze the collective "spatial" and temporal dynamics of companies in nine sectors of the world economy over a 19 year period from 1990-2008. These metrics provide accurate predictive skill with a very low false-positive rate in predicting downturns. The new metrics also provide evidence of phase transition-like behavior prior to the onset of recessions. Such a transition occurs when negative pretax incomes prior to or during economic recessions transition from a thin-tailed exponential distribution to the higher entropy Pareto distribution, and develop even heavier tails than those of the positive pretax incomes. These features propagate from the crisis initiating sector of the economy to other sectors.
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The Uses of Crisis: Taking the Tide at the Flood.
ERIC Educational Resources Information Center
Kerchner, Charles T.; Schuster, Jack H.
1982-01-01
Crises can, under certain conditions, be transformed into instruments of organizational good. Undergoing crisis in an organization--calling attention to problems and labeling them as crises--may be a preferable management strategy to coping with prolonged periods of shrinking resources. (MLW)
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Raven, Joanna; Baral, Sushil; Wurie, Haja; Witter, Sophie; Samai, Mohamed; Paudel, Pravin; Subedi, Hom Nath; Martineau, Tim; Elsey, Helen; Theobald, Sally
2018-02-07
Health workers are critical to the performance of health systems; yet, evidence about their coping strategies and support needs during and post crisis is lacking. There is very limited discussion about how research teams should respond when unexpected crises occur during on-going research. This paper critically presents the approaches and findings of two health systems research projects that explored and evaluated health worker performance and were adapted during crises, and provides lessons learnt on re-orientating research when the unexpected occurs. Health systems research was adapted post crisis to assess health workers' experiences and coping strategies. Qualitative in-depth interviews were conducted with 14 health workers in a heavily affected earthquake district in Nepal and 25 frontline health workers in four districts in Ebola-affected Sierra Leone. All data were transcribed and analysed using the framework approach, which included developing coding frameworks for each study, applying the frameworks, developing charts and describing the themes. A second layer of analysis included analysis across the two contexts, whereas a third layer involved the research teams reflecting on the approaches used to adapt the research during these crises and what was learned as individuals and research teams. In Sierra Leone, health workers were heavily stigmatised by the epidemic, leading to a breakdown of trust. Coping strategies included finding renewed purpose in continuing to serve their community, peer and family support (in some cases), and religion. In Nepal, individual determination, a sense of responsibility to the community and professional duty compelled staff to stay or return to their workplace. The research teams had trusting relationships with policy-makers and practitioners, which brought credibility and legitimacy to the change of research direction as well as the relationships to maximise the opportunity for findings to inform practice. In both contexts, health workers demonstrated considerable resilience in continuing to provide services despite limited support. Embedded researchers and institutions are arguably best placed to navigate emerging ethical and social justice challenges and are strategically positioned to support the co-production of knowledge and ensure research findings have impact.
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NASA Astrophysics Data System (ADS)
Gregg, C. E.; Reeves, A.; Lindell, M. K.; Prater, C.; Joyner, T. A.; Eggert, S.
2016-12-01
The eruption of Kīlauea volcano since 1983 has produced a series of crises, the latest one occurring in 2014 and 2015 when a new vent sent lava flows northeastward toward developed areas in the lower Puna District of Kīlauea. The June 27 lava flow took about 2 months to advance to the edge of developed areas in Puna, prompting widespread reaction. Volcanic eruptions often have large economic consequences out of proportion with their magnitudes, and uncertainties about the physical and organizational communication of risk information amplify these losses. This study aims to improve tools to communicate uncertainty of volcanic activity and organizational and individual response, offering clearer and more reliable information to guide civic leaders in issuing appropriate warnings. One significant impediment to risk communication is limited knowledge about the most effective ways to communicate scientific uncertainty through verbal, numeric and graphic methods. The public's demand for near-real time information updates during the June 27 lava crisis, including both written messages and graphics, required some agencies to provide information at a faster rate than in any previous eruption. In order to understand how these and other stakeholders involved with the crisis can better plan for and manage future crises, including implementing evacuation decisions, we conducted a series of interviews and a mental model exercise with stakeholders. We explored their knowledge of local risk communication messages and hazard mitigation efforts and their experiences during the June 27 lava flow crisis. Stakeholders represented county, state and federal agencies and included elected officials, emergency managers, scientists, and other professionals involved with the crisis (traffic engineers, land use planners, police officers, fire fighters). We also assessed factors that influence individual and household preparedness to implement officials' protective action recommendations, such as evacuation, and their attitudes toward hazard mitigation efforts. Collectively, these two studies provide a detailed evaluation of important risk communication and risk management issues at both individual and organizational levels and insight about uncertainties that influence the outcome of volcanic crises.
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Debray, François-Guillaume; Morin, Charles; Janvier, Annie; Villeneuve, Josée; Maranda, Bruno; Laframboise, Rachel; Lacroix, Jacques; Decarie, Jean-Claude; Robitaille, Yves; Lambert, Marie; Robinson, Brian H; Mitchell, Grant A
2011-03-01
The natural history of all known patients with French-Canadian Leigh disease (Saguenay-Lac-St-Jean cytochrome c oxidase deficiency, MIM220111, SLSJ-COX), the largest known cohort of patients with a genetically homogeneous, nuclear encoded congenital lactic acidosis, was studied. 55 of 56 patients were homozygous for the A354V mutation in LRPPRC. One was a genetic compound (A354V/C1277Xdel8). Clinical features included developmental delay, failure to thrive, characteristic facial appearance and, in 90% of patients, acute crises that have not previously been detailed, either metabolic (fulminant lactic acidosis) and/or neurological (Leigh syndrome and/or stroke-like episodes). Survival ranged from 5 days to >30 years. 46/56 patients (82%) died, at a median age of 1.6 years. Of 73 crises, 38 (52%) were fatal. The immediate causes of death were multiple organ failure and/or Leigh disease. Major predictors of mortality during crises (p<0.005) were hyperglycaemia, hepatic cytolysis, and altered consciousness at admission. Compared to a group of SURF1-deficient Leigh syndrome patients assembled from the literature, SLSJ-COX is distinct by the occurrence of metabolic crises, leading to earlier and higher mortality (p=0.001). SLSJ-COX is clinically distinct, with acute fatal acidotic crises on a backdrop of chronic moderate developmental delay and hyperlactataemia. Leigh syndrome is common. Stroke-like episodes can occur. The Leigh syndrome of SLSJ-COX differs from that of SURF1-related COX deficiency. SLSJ-COX has a different spectrum of associated abnormalities, acidotic crises being particularly suggestive of LRPPRC related Leigh syndrome. Even among A354V homozygotes, pronounced differences in survival and severity occur, showing that other genetic and/or environmental factors can influence outcome.
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Employee Communication during Crises: The Effects of Stress on Information Processing.
ERIC Educational Resources Information Center
Pincus, J. David; Acharya, Lalit
Based on multidisciplinary research findings, this report proposes an information processing model of employees' response to highly stressful information environments arising during organizational crises. The introduction stresses the importance of management's handling crisis communication with employees skillfully. The second section points out…
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ERIC Educational Resources Information Center
Schultz, Beatrice, Ed.
1986-01-01
Topics discussed include: how men and women conceive and manage crises in romantic relationships; differences in how men and women handle crises in an organizational setting; the common talk men make about their relationships; the forces that shape women's relationships with men; and the priorities for future studies. (RM)
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Episodic processes, invasion and faunal mosaics in evolutionary and ecological time
USDA-ARS?s Scientific Manuscript database
Episodes of ecological perturbation and faunal turnover represent crises for global biodiversity and have occurred periodically across Earth history on a continuum linking deep evolutionary and shallow ecological time. Major extinction events and biodiversity crises across the 540 milion years of th...
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Mental Health Aspects of Responding to Campus Crises
ERIC Educational Resources Information Center
Flynn, Christopher; Sharma, Micky M.
2016-01-01
Tragedy can strike a college campus in unpredictable and often horrific ways that may lead to traumatic responses for individuals and the entire campus community. Crises on campus demand an appropriate response to support the community, provide assistance to affected individuals and guide healing efforts.
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Winter, David G
2007-05-01
Drawing on D. G. Winter's (1993) comparison of 1914 and the Cuban Missile Crisis, the author identified 8 paired crises (1 escalating to war, 1 peacefully resolved). Documents (diplomatic messages, speeches, official media commentary) from each crisis were scored for power, affiliation, and achievement motivation; text measures of responsibility and activity inhibition; and integrative complexity. Aggregated effect-size results show that war crises had significantly higher levels of power motivation and responsibility, whereas peace crises showed trends toward higher integrative complexity and achievement motivation. Follow-up analyses suggested that these results are robust with respect to both sides in a crisis, type of material scored, and historical time. The power motive results extend previous findings, but the responsibility results suggest that responsibility plays a paradoxical role in war. Future research directions are sketched, and the role of psychological content analysis in monitoring the danger of war is discussed. ((c) 2007 APA, all rights reserved).
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Assessment of economic vulnerability to infectious disease crises.
Sands, Peter; El Turabi, Anas; Saynisch, Philip A; Dzau, Victor J
2016-11-12
Infectious disease crises have substantial economic impact. Yet mainstream macroeconomic forecasting rarely takes account of the risk of potential pandemics. This oversight contributes to persistent underestimation of infectious disease risk and consequent underinvestment in preparedness and response to infectious disease crises. One reason why economists fail to include economic vulnerability to infectious disease threats in their assessments is the absence of readily available and digestible input data to inform such analysis. In this Viewpoint we suggest an approach by which the global health community can help to generate such inputs, and a framework to use these inputs to assess the economic vulnerability to infectious disease crises of individual countries and regions. We argue that incorporation of these risks in influential macroeconomic analyses such as the reports from the International Monetary Fund's Article IV consultations, rating agencies and risk consultancies would simultaneously improve the quality of economic risk forecasting and reinforce individual government and donor incentives to mitigate infectious disease risks. Copyright © 2016 Elsevier Ltd. All rights reserved.
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Stickler, D. J.; Jones, S. M.; Adusei, G. O.; Waters, M. G.
2006-01-01
A simple sensor has been developed to detect the early stages of urinary catheter encrustation and avoid the clinical crises induced by catheter blockage. In laboratory models of colonization by Proteus mirabilis, the sensor signaled encrustation at an average time of 43 h before catheters were blocked with crystalline biofilm. PMID:16597888
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Code of Federal Regulations, 2012 CFR
2012-01-01
... developing world and the American people. Since President John F. Kennedy founded USAID in 1961, the men and... countries as they build a better future. By promoting sustainable growth in the developing world, we spur... national security. And by delivering aid in the wake of natural disasters and humanitarian crises, we...
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Insomnia and Psychosocial Crisis: Two Studies of Erikson's Developmental Theory.
ERIC Educational Resources Information Center
Wagner, Karen Dineen; And Others
1983-01-01
Examines the role of internal stressors in the development of sleep disturbances in two studies of 122 older adults and 66 college students. Results confirmed Erikson's (1959) developmental theory. Failure to resolve the psychosocial crises of old age and adolescence were related to insomnia. (WAS)
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Emotional First Aid: Crisis Development and Systems of Intervention.
ERIC Educational Resources Information Center
Rosenbluh, Edward S.; And Others
This instructional manual takes a developmental approach toward understanding the psychological, social and behavioral dynamics of human crisis. The manual describes the behavior patterns characterizing various psychological and physical crises, and provides background information and methods of crisis intervention with which to manage each. In…
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NASA Astrophysics Data System (ADS)
Sobradelo, Rosa; Martí, Joan; Kilburn, Christopher; López, Carmen
2014-05-01
Understanding the potential evolution of a volcanic crisis is crucial to improving the design of effective mitigation strategies. This is especially the case for volcanoes close to densely-populated regions, where inappropriate decisions may trigger widespread loss of life, economic disruption and public distress. An outstanding goal for improving the management of volcanic crises, therefore, is to develop objective, real-time methodologies for evaluating how an emergency will develop and how scientists communicate with decision makers. Here we present a new model BADEMO (Bayesian Decision Model) that applies a general and flexible, probabilistic approach to managing volcanic crises. The model combines the hazard and risk factors that decision makers need for a holistic analysis of a volcanic crisis. These factors include eruption scenarios and their probabilities of occurrence, the vulnerability of populations and their activities, and the costs of false alarms and failed forecasts. The model can be implemented before an emergency, to identify actions for reducing the vulnerability of a district; during an emergency, to identify the optimum mitigating actions and how these may change as new information is obtained; and after an emergency, to assess the effectiveness of a mitigating response and, from the results, to improve strategies before another crisis occurs. As illustrated by a retrospective analysis of the 2011 eruption of El Hierro, in the Canary Islands, BADEMO provides the basis for quantifying the uncertainty associated with each recommended action as an emergency evolves, and serves as a mechanism for improving communications between scientists and decision makers.
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Fernandez-Luque, Luis; Imran, Muhammad
2018-06-01
According to the World Health Organization (WHO), over 130 million people are in constant need of humanitarian assistance due to natural disasters, disease outbreaks, and conflicts, among other factors. These health crises can compromise the resilience of healthcare systems, which are essential for achieving the health objectives of the sustainable development goals (SDGs) of the United Nations (UN). During a humanitarian health crisis, rapid and informed decision making is required. This is often challenging due to information scarcity, limited resources, and strict time constraints. Moreover, the traditional approach to digital health development, which involves a substantial requirement analysis, a feasibility study, and deployment of technology, is ill-suited for many crisis contexts. The emergence of Web 2.0 technologies and social media platforms in the past decade, such as Twitter, has created a new paradigm of massive information and misinformation, in which new technologies need to be developed to aid rapid decision making during humanitarian health crises. Humanitarian health crises increasingly require the analysis of massive amounts of information produced by different sources, such as social media content, and, hence, they are a prime case for the use of artificial intelligence (AI) techniques to help identify relevant information and make it actionable. To identify challenges and opportunities for using AI in humanitarian health crises, we reviewed the literature on the use of AI techniques to process social media. We performed a narrative literature review aimed at identifying examples of the use of AI in humanitarian health crises. Our search strategy was designed to get a broad overview of the different applications of AI in a humanitarian health crisis and their challenges. A total of 1459 articles were screened, and 24 articles were included in the final analysis. Successful case studies of AI applications in a humanitarian health crisis have been reported, such as for outbreak detection. A commonly shared concern in the reviewed literature is the technical challenge of analyzing large amounts of data in real time. Data interoperability, which is essential to data sharing, is also a barrier with regard to the integration of online and traditional data sources. Human and organizational aspects that might be key factors for the adoption of AI and social media remain understudied. There is also a publication bias toward high-income countries, as we identified few examples in low-income countries. Further, we did not identify any examples of certain types of major crisis, such armed conflicts, in which misinformation might be more common. The feasibility of using AI to extract valuable information during a humanitarian health crisis is proven in many cases. There is a lack of research on how to integrate the use of AI into the work-flow and large-scale deployments of humanitarian aid during a health crisis. Copyright © 2018 Elsevier B.V. All rights reserved.
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A model of international financial crises
NASA Astrophysics Data System (ADS)
Kaizoji, Taisei
2001-10-01
This paper proposes a model of international financial crises that is based on the statistical mechanics. In our model the international stock market is composed of two groups of traders mutually influencing each other with respect to their decision behavior, and financial contagion between markets occurs as a result of attempts by traders in the domestic market to imitate the behavior of traders who participate into exchange in a foreign market. This provides a channel through which a crisis in one market such as contemporaneous stock market crashes can be transmitted to other markets. We show that the model can explain the stylized facts characterizing periods of recent international financial crises.
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Suedfeld, Peter; Soriano, Erin; McMurtry, Donna Louise; Paterson, Helen; Weiszbeck, Tara L; Krell, Robert
2005-01-01
This study assessed the degree to which Holocaust survivors have dealt successfully with the eight psychosocial crises thought by Erikson (1959) to mark important stages in life-span development. In Study 1, 50 autobiographical interviews of survivors videotaped 30-50 years after the war were subjected to thematic content analysis. Relevant passages were coded as representing either a favorable or an unfavorable outcome as defined by Erikson. Survivors described significantly more favorable than unfavorable outcomes for seven of the crises; the exception was Trust vs. Mistrust. In Study 2, audiotaped Holocaust survivor interviews conducted in 1946 were scored in the same way and compared with the results of Study 1. There were several significant differences as well as similarities between the two data sets, the later interviews mostly showing changes in the positive direction.
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An Auto-Ethnographic Study of "Open Dialogue": The Illumination of Snow.
Olson, Mary
2015-12-01
This auto-ethnographic study describes the changes in the author's thinking and clinical work connected to her first-hand experience of Open Dialogue, which is an innovative, psychosocial approach to severe psychiatric crises developed in Tornio, Finland. In charting this trajectory, there is an emphasis on three interrelated themes: the micropolitics of U.S. managed mental health care; the practice of "dialogicality" in Open Dialogue; and the historical, cultural, and scientific shifts that are encouraging the adaptation of Open Dialogue in the United States. The work of Gregory Bateson provides a conceptual framework that makes sense of the author's experience and the larger trends. The study portrays and underscores how family and network practices are essential to responding to psychiatric crises and should not be abandoned in favor of a reductionist, biomedical model. © 2015 Family Process Institute.
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Community Crisis Intervention; A Training Manual.
ERIC Educational Resources Information Center
Batson, Ruth; Peters, Lyda
This manual is designed to help community mental health workers to intervene effectively in community crises, particularly school desegregation. The rationale for involving mental health personnel in community crises is explained. The need for expertise among such workers is emphasized, and strategies for gaining this expertise are outlined. Also…
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Intercultural Competence in Education, Counselling and Psychotherapy
ERIC Educational Resources Information Center
Portera, Agostino
2014-01-01
The present era of globalization, interdependence and multicultural societies has brought about both opportunities and crises in educational institutions. Especially schools and families seem to be unable to cope with such revolutionary changes. The crises also concern all fields involved in maintaining social welfare, including counselling and…
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NASA Astrophysics Data System (ADS)
Mayberry, G. C.; Pallister, J. S.
2015-12-01
The Volcano Disaster Assistance Program (VDAP) is a joint effort between USGS and the U.S. Agency for International Development's (USAID) Office of U.S. Foreign Disaster Assistance (OFDA). OFDA leads and coordinates disaster responses overseas for the U.S. government and is a unique stakeholder concerned with volcano disaster risk reduction as an international humanitarian assistance donor. One year after the tragic eruption of Nevado del Ruiz in 1985, OFDA began funding USGS to implement VDAP. VDAP's mission is to reduce the loss of life and property and limit the economic impact from foreign volcano crises, thereby preventing such crises from becoming disasters. VDAP fulfills this mission and complements OFDA's humanitarian assistance by providing crisis response, capacity-building, technical training, and hazard assessments to developing countries before, during, and after eruptions. During the past 30 years, VDAP has responded to more than 27 major volcanic crises, built capacity in 12+ countries, and helped counterparts save tens of thousands of lives and hundreds of millions of dollars in property. VDAP responses have evolved as host-country capabilities have grown, but the pace of work has not diminished; as a result of VDAP's work at 27 volcanoes in fiscal year 2014, more than 1.3 million people who could have been impacted by volcanic activity benefitted from VDAP assistance, 11 geological policies were modified, 188 scientists were trained, and several successful eruption forecasts were made. VDAP is developing new initiatives to help counterparts monitor volcanoes and communicate volcanic risk. These include developing the Eruption Forecasting Information System (EFIS) to learn from compiled crisis data from 30 years of VDAP responses, creating event trees to forecast eruptions at restless volcanoes, and exploring the use of unmanned aerial systems for monitoring. The use of these new methods, along with traditional VDAP assistance, has improved VDAP's ability to assist counterparts with preparing for eruptions.
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Characterization of anemia induced by avian osteopetrosis virus.
Paterson, R W; Smith, R E
1978-01-01
Chickens infected intravenously at 8 days after hatching with an avian osteopetrosis virus developed a severe, progressive anemia in the absence of osteopetrosis. The anemia was characterized as a pancytopenia, in which erythrocytes, granulocytes, and thrombocytes decreased concomitantly. Serum bilirubin levels were normal, whereas erythrocytes from infected chickens demonstrated a slightly elevated osmotic fragility. A negative Coombs test indicated that there was no evidence for erythrocyte-bound antibody. Erythrocytes from infected animals had slightly decreased 51Cr-labeled erythrocyte survival time when compared with normal. Examination of marrow histological preparations, together with ferrokinetic studies with 59Fe, indicated that marrow failure occurred during the acute phase of the anemia. Circulating virus was present during the development and acute phases of the anemia, but disappeared during the recovery phase of the disease. Neutralizing antibody appeared after the disappearance of circulating virus. It is concluded that virus infection induced both marrow failure (aplastic crisis) and decreased erythrocyte survival. Images PMID:215554
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CRADAs: They're Not Just for NCI Anymore | Poster
By Karen Surabian, Thomas Stackhouse, and Jeffrey Thomas, Contributing Writers, and Bruce Crise, Guest Writer Advancing scientific discovery is increasingly dependent on diverse and innovative partnerships, and the Cooperative Research and Development Agreement (CRADA) is an essential tool for establishing partnerships. CRADAs allow a federal laboratory to enter into
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Building the Case for Educating Business Leaders on the Importance of Public Relations
ERIC Educational Resources Information Center
Hagan, Linda M.
2011-01-01
An organization's well-developed strategic plan and reputation can crumble in minutes if it mishandles key communications, particularly during crises. Today's leaders need to appreciate the value of professional public relations and how it helps organizational effectiveness by building strategic relationships, maintaining a favorable reputation,…
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7 CFR 1486.209 - How are program applications evaluated and approved?
Code of Federal Regulations, 2013 CFR
2013-01-01
... emerging markets such as activities intended to mitigate the impact of sudden political events or economic and currency crises in order to maintain U.S. market share; (ii) Marketing and distribution of value-added products, including new products or new uses. Examples include food service development, market...
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7 CFR 1486.209 - How are program applications evaluated and approved?
Code of Federal Regulations, 2014 CFR
2014-01-01
... emerging markets such as activities intended to mitigate the impact of sudden political events or economic and currency crises in order to maintain U.S. market share; (ii) Marketing and distribution of value-added products, including new products or new uses. Examples include food service development, market...
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7 CFR 1486.209 - How are program applications evaluated and approved?
Code of Federal Regulations, 2012 CFR
2012-01-01
... emerging markets such as activities intended to mitigate the impact of sudden political events or economic and currency crises in order to maintain U.S. market share; (ii) Marketing and distribution of value-added products, including new products or new uses. Examples include food service development, market...
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Growing Pains: A Theory of Stress and Moral Conflict.
ERIC Educational Resources Information Center
Blakeney, Ronnie Ann; Blakeney, Charles David
1992-01-01
Outlines relationship between crises that create stress in one's life and periods of instability in moral development. Suggests that stressful issues are more difficult to resolve during periods of developmental instability and that, when problems are most stressful, people often experience impact of stress physiologically. Reconstructs stress and…
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Trial by Fire (and Tornado) Taught Us to Plan for Crises.
ERIC Educational Resources Information Center
Caylor, Mary Jane
1991-01-01
Based on Huntsville (Alabama) schools' experience with a devastating fire, the superintendent later ensured adequate fire insurance coverage, promoted regular fire drills, and developed an emergency response plan that delineated staff responsibilities, communication modes, and training and updating procedures. The plan served the district well…
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Risk Management and Crisis Response: Are You Prepared?
ERIC Educational Resources Information Center
Schirick, Ed
2002-01-01
How a camp responds to a crisis may determine whether it can survive financially. Effective risk management requires total commitment from ownership and management, and staff involvement. Steps in formulating a risk management plan include identifying all potential crises and their frequency and severity potential, developing responses,…
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Environmental Literacy of Hispanic, Urban, Middle School Students in Houston, Texas
ERIC Educational Resources Information Center
Meuth, Amber M.
2010-01-01
With the global crises facing the planet that bring major implications, (Hart & Nolan, 1999; Hungerford & Simmons, 2003) it is imperative that there be an environmentally literate citizenry who can identify, solve, and prevent environmental issues. Since middle school students are evolving into participating citizens and are developing the…
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ERIC Educational Resources Information Center
Ecclestone, Kathryn; Goodley, Daniel
2016-01-01
Contemporary discourses of social justice in education, disability, mental health, social policy and feminist studies are refracted increasingly through concerns about psychological and structural vulnerabilities created by the crises of late capitalism. Focusing on developments in British social policy generally and educational research…
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Geriatrix: A Role-Playing Game.
ERIC Educational Resources Information Center
Hoffman, Stephanie B.; And Others
1985-01-01
To sensitize medical students to the complexities of geriatric patient care, the geriatrics faculty of Upstate Medical Center's Clinical Campus developed Geriatrix, a role-playing game. Students attempt to win points in a board game containing Fact, Concept, and Crisis Squares. Crises promote exposure to ethical dilemmas and invite intense debate…
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Coaching Techniques and Play Therapy Responses: A New Communication Pattern
ERIC Educational Resources Information Center
Muro, Joel H.; Stulmaker, Hayley; Rose, Katherine Kensinger
2012-01-01
Understanding the developmental stages of children is crucial for coaches. Equally important is the way adults communicate with children. In his seminal work describing the influence of society and social interactions on the psychosocial development of children, Erikson (1950, 1963) described the developmental crises faced by children at each…
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School Mental Health's Response to Terrorism and Disaster.
ERIC Educational Resources Information Center
Weist, Mark D.; Sander, Mark A.; Lever, Nancy A.; Rosner, Leah E.; Pruitt, David B.; Lowie, Jennifer Axelrod; Hill, Susan; Lombardo, Sylvie; Christodulu, Kristin V.
2002-01-01
Explores the response of school mental health to terrorism and disaster, reviewing literature on child and adult reactions to trauma, discussing the development of crisis response teams, and presenting strategies for schools to respond to crises and disaster. One elementary school's experiences in response to the September 11th attacks are…
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Rebooting Irish Higher Education: Policy Challenges for Challenging Times
ERIC Educational Resources Information Center
Hazelkorn, Ellen
2014-01-01
The 2008 global financial crisis cast a long shadow over Ireland's higher education and research system. The IMF said Ireland experienced an "unprecedented economic correction", while Ireland's National Economic and Social Development Office said Ireland was beset by five different crises: a banking crisis, a fiscal crisis, an economic…
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Chairman of Joint Chiefs of Staff, 7th Annual Seminar on Peace Operations
2001-07-01
we CSL - 2 need to be proactive toward managing crises rather than reactive. He also highlighted the many dimensions to these complex global ... issues -- political, economic and military. Interagency members must avoid a myopic view of the problem. The interagency team must develop and
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Three Campus Crises: Lessons for Administrators.
ERIC Educational Resources Information Center
Rothman, Michael
1992-01-01
In October 1989-October 1991, Mills College (California) faced three crises: an earthquake; a strike over plans to convert to a coeducational institution; and a devastating fire in the area. The college has learned to focus its structural, human, political, and symbolic frames of reference; communicate; plan; consider the public; and develop…
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Campus Communications in the Age of Crises
ERIC Educational Resources Information Center
McGuire, Patricia
2007-01-01
Recent catastrophes have brought about numerous critiques and changes to campus communications. In this article, the author shares the lessons she has learned from the crises she experienced during her 18 years of being the president of Trinity (Washington) University. Furthermore, Joan Hinde Stewart, president of Hamilton College, adds her…
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ERIC Educational Resources Information Center
Futurist, 1984
1984-01-01
Five areas that have great potential for becoming crises in the future are described: a warming of the earth's climate, changing weather patterns and growing seasons; water shortage; the decay of the physical infrastructure, e.g., decay of roads, bridges; breakdown of the international monetary and trading system; and nuclear warfare. (Author/RM)
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Preparing for School Crises: Administrator Perceptions on Supports for Teachers
ERIC Educational Resources Information Center
Brophy, Chelsey M.; Maras, Melissa A.; Wang, Ze
2015-01-01
Traumatic events and crises involving schools and children often become high-profile occurrences; however, little attention is given to teachers and how they cope with crisis. The purpose of this study was to investigate administrators' perceptions of including additional support for teachers in school crisis policies. Specifically, the study…
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ERIC Educational Resources Information Center
Zipin, Lew; Brennan, Marie
2003-01-01
"Fiscal" and other so-called "crises" in Australian universities are more fundamentally, it is argued in this article, crises of government decision and "governmentality". Using an illustrative "morality tale" drawn from their working knowledge of the Australian university sector, the authors take a…
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ERIC Educational Resources Information Center
Evans, Tina Lynn
2015-01-01
This article takes as its point of departure the many converging crises of sustainability and the responsibility of higher education institutions and faculty members to participate in mitigating these crises to any extent possible. The author characterizes sustainability education as transdisciplinary praxis, explores the institutional and…
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CRADAs: They're Not Just for NCI Anymore | Poster
By Karen Surabian, Thomas Stackhouse, and Jeffrey Thomas, Contributing Writers, and Bruce Crise, Guest Writer Advancing scientific discovery is increasingly dependent on diverse and innovative partnerships, and the Cooperative Research and Development Agreement (CRADA) is an essential tool for establishing partnerships. CRADAs allow a federal laboratory to enter into collaborative research and development (R&D) projects with outside parties (commercial or nonprofit).
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Panel 2.17: private commercial sector partnerships for health action in crises.
Sundnes, Knut Ole; Sannerkvist, Milan; Hedger, Philip; Woodworth, Brent; Hyre, Anne; Cuddyre, Terrence; Waldman, Ronald
2005-01-01
This is a summary of the presentations and discussion of Panel 2.17, Private Commercial Sector Partnerships for Health Action in Crises of the Conference, Health Aspects of the Tsunami Disaster in Asia, convened by the World Health Organization (WHO) in Phuket, Thailand, 04-06 May 2005. The topics discussed included issues related to private sector partnerships for health action in crises as pertain to the responses to the damage created by the Tsunami. It is presented in the following sections: (1) key questions; (2) issues and challenges; (3) lessons learned; (4) what was done well?; (5) what could have been done better?; and (6) conclusions and recommendations.
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Potentially preventable hospitalizations in dementia: family caregiver experiences.
Sadak, Tatiana; Foster Zdon, Susan; Ishado, Emily; Zaslavsky, Oleg; Borson, Soo
2017-07-01
Health crises in persons living with dementia challenge their caregivers to make pivotal decisions, often under pressure, and to act in new ways on behalf of their care recipient. Disruption of everyday routines and heightened stress are familiar consequences of these events. Hospitalization for acute illness or injury is a familiar health crisis in dementia. The focus of this study is to describe the lived experience of dementia family caregivers whose care recipients had a recent unplanned admission, and to identify potential opportunities for developing preventive interventions. Family caregivers (n = 20) of people with dementia who experienced a recent hospitalization due to an ambulatory care sensitive condition or fall-related injury completed phone interviews. Interviews used semi-structured protocols to elicit caregivers' reactions to the hospitalization and recollections of the events leading up to it. Analysis of interview data identified four major themes: (1) caregiver is uncertain how to interpret and act on the change; (2) caregiver is unable to provide necessary care; (3) caregiver experiences a personal crisis in response to the patient's health event; (4) mitigating factors may prevent caregiver crises. This study identifies a need for clinicians and family caregivers to work together to avoid health crises of both caregivers and people with dementia and to enable caregivers to manage the health of their care recipients without sacrificing their own health and wellness.
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The Nigerian State and Global Economic Crises: Socio-Political Implications and Policy Challenges
ERIC Educational Resources Information Center
Olaopa, O. R.; Ogundari, I. O.; Akindele, S. T.; Hassan, O. M.
2012-01-01
This article discusses how economic reforms, as a reaction to the effects of the global financial crises, have intensified popular unrests and redefined the composition, interests, and socio-economic and political attitudes of Nigeria's increasingly complex social strata. We relied basically on secondary data to analyze some of the fundamental…
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Risks and crises for healthcare providers: the impact of cloud computing.
Glasberg, Ronald; Hartmann, Michael; Draheim, Michael; Tamm, Gerrit; Hessel, Franz
2014-01-01
We analyze risks and crises for healthcare providers and discuss the impact of cloud computing in such scenarios. The analysis is conducted in a holistic way, taking into account organizational and human aspects, clinical, IT-related, and utilities-related risks as well as incorporating the view of the overall risk management.
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Before Crisis Hits: Building a Strategic Crisis Plan.
ERIC Educational Resources Information Center
Smith, Larry L.; Millar, Dan P.
This guide offers suggestions to college administrators for dealing with a variety of emergency or crisis situations that could affect a community college's effectiveness. The authors used the Institute for Crisis Management's (ICM) four types of crises in higher education as the framework for the guide. The four types of crises are: (1) sudden;…
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DOE Office of Scientific and Technical Information (OSTI.GOV)
Jeffries, C.; Perez, J.
For a driven nonlinear oscillator we report direct evidence for three cases of an interior crisis of the attractor, as conjectured by Grebogi, Ott, and Yorke. These crises are sudden and discontinuous changes in the attractor, observed directly from bifurcation diagrams and attractor diagrams (Poincare sections) in real time. The crises arise from intersection of an unstable orbit with the chaotic attractor.
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Planning for a Better Future: California 2025. 2011 Update
ERIC Educational Resources Information Center
Public Policy Institute of California, 2011
2011-01-01
California's current economic and fiscal realities make nonpartisan, objective information on the state's future challenges all the more critical. Understandably, the search is on for immediate solutions to the unprecedented crises people face today. But if the present crises make policymakers shelve long-term planning, the result may be an even…
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2017-01-01
13 Maritime safety ...deterrence, ballistic missile defense, deterrence of conventional crises and war (through naval readiness and engagement), maritime safety operations...confidence-building measure and to avoid unwanted crises.55 Maritime safety This an area that is primarily the domain of the world’s Coast Guards
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ERIC Educational Resources Information Center
Leuschner, Vincenz; Fiedler, Nora; Schultze, Martin; Ahlig, Nadine; Göbel, Kristin; Sommer, Friederike; Scholl, Johanna; Cornell, Dewey; Scheithauer, Herbert
2017-01-01
The standardized, indicated school-based prevention program "Networks Against School Shootings" combines a threat assessment approach with a general model of prevention of emergency situations in schools through early intervention in student psychosocial crises and training teachers to recognize warning signs of targeted school violence.…
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Marketing Crises in Tourism: Communication Strategies in the United States and Spain.
ERIC Educational Resources Information Center
Gonzalez-Herrero, Alfonso; Pratt, Cornelius B.
1998-01-01
Compares crisis-response strategies of marketing-communication professionals in tourism organizations (TOs) in the United States and Spain. Reports the extent to which they use proven crisis-management strategies. Indicates significant differences between the countries' TOs in both their extant plans for responding to marketing crises and in their…
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Crises and Opportunities: The Futures of Scholarly Publishing. ACLS Occasional Paper, No. 57
ERIC Educational Resources Information Center
Alonso, Carlos J.; Davidson, Cathy N.; Unsworth, John M.; Withey, Lynne
2003-01-01
Presented herein are papers presented at a session entitled "Crises and Opportunities: The Future of Scholarly Publishing," from the American Council of Learned Societies (ACLS) Annual Meeting, May 10, 2003. Four speakers approached this topic from different standpoints: as leaders of learned societies, as senior university officials, from the…
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Summoning Spectres: Crises and Their Construction
ERIC Educational Resources Information Center
Clarke, John; Newman, Janet
2010-01-01
The construction of crises is a key analytical and political issue. This paper examines what is at stake in the processes and practices of construction, responding to the arguments made in Andrew Gamble's "The spectres at the feast" (2009). We suggest that there are three areas of critical concern: first, that too little attention has…
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Supporting Students with Disabilities during School Crises: A Teacher's Guide
ERIC Educational Resources Information Center
Clarke, Laura S.; Embury, Dusty Columbia; Jones, Ruth E.; Yssel, Nina
2014-01-01
Most schools have crisis plans to support student safety, but few plans address the complex needs of students with disabilities. School supports should include analysis of school plans and student strengths and needs to ensure that students with disabilities have the best opportunity to be safe in school crises. Recommendations include developing…
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Recovery from Large-Scale Crises: Guidelines for Crisis Teams and Administrators
ERIC Educational Resources Information Center
Communique, 2018
2018-01-01
The United States has recently experienced numerous large-scale crises that resulted in high death tolls and extensive property damage. Critical incidents such as suicide contagion, natural disasters, or mass casualty events have the potential to cause traumatic reactions and significantly affect children's sense of safety and security. School…
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Using Crises, Feedback, and Fading for Online Task Design
ERIC Educational Resources Information Center
Bokhove, Christian
2014-01-01
A recent discussion involves the elaboration on possible design principles for sequences of tasks. This paper builds on three principles, as described by Bokhove and Drijvers (2012a). A model with ingredients of crises, feedback and fading of sequences with near-similar tasks can be used to address both procedural fluency and conceptual…
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The Crises and Freedoms of Researching Your Own Life
ERIC Educational Resources Information Center
Pearce, Caroline
2010-01-01
There has been much work highlighting the benefits of autoethnographic research yet little acknowledgement of the demands researching your own life makes on the emotional and mental wellbeing of the researcher. This paper explores the consequences that can arise as a result of autoethnographic research by detailing the crises involved in…
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Smith, James; Roberts, Bayard; Knight, Abigail; Gosselin, Richard; Blanchet, Karl
2015-11-01
Humanitarian crises continue to pose a significant threat to health; the United Nations estimates that 144 million people are directly affected by conflict or environmental disasters. During most humanitarian crises, surgical and rehabilitative interventions remain a priority. This review assessed the quality of evidence that informs injury and physical rehabilitation interventions in humanitarian crises. Peer-reviewed and grey literature sources were assessed in a systematic manner. Selected papers were evaluated using quality criteria based on a modified version of the STROBE protocol. 46 papers met the inclusion criteria. 63 % of the papers referred to situations of armed conflict, of which the Yugoslav Wars were the most studied crisis context. 59 % of the studies were published since the year 2000. However, only two studies were considered of a high quality. While there is now a greater emphasis on research in this sector, the volume of evidence remains inadequate given the growing number of humanitarian programmes worldwide. Further research is needed to ensure a greater breadth and depth of understanding of the most appropriate interventions in different settings.
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Oral cancer in Fanconi anemia: Review of 121 cases.
Furquim, Camila Pinheiro; Pivovar, Allana; Amenábar, José Miguel; Bonfim, Carmem; Torres-Pereira, Cassius Carvalho
2018-05-01
Fanconi anemia (FA) is a rare autosomal recessive genetic disorder characterized by aplastic anemia, progressive pancytopenia, congenital anomalies, and increased risk of cancer development. After hematopoietic stem cell transplant (HSCT), patients have an estimated 500-fold increase in the risk of developing head and neck cancer compared to a non-affected, and the oral cavity is affected in one-third of cases. Thus, this study aimed to better understand the natural history of oral cavity cancer in patients affected by FA. After conducting a keyword search on MEDLINE, we found 121 cases of oral cavity cancer in patients who had been affected by FA. In conclusion, HSCT may increase the risks of oral cancer development, especially after 5 years after the transplant. In the normal population, the tongue is the most affected area. FA patients should be informed of the risks of oral malignant transformation and encouraged to be undergo medical surveillance. Copyright © 2018 Elsevier B.V. All rights reserved.
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Telomere-driven diseases and telomere-targeting therapies
2017-01-01
Telomeres, the protective ends of linear chromosomes, shorten throughout an individual’s lifetime. Telomere shortening is proposed to be a primary molecular cause of aging. Short telomeres block the proliferative capacity of stem cells, affecting their potential to regenerate tissues, and trigger the development of age-associated diseases. Mutations in telomere maintenance genes are associated with pathologies referred to as telomere syndromes, including Hoyeraal-Hreidarsson syndrome, dyskeratosis congenita, pulmonary fibrosis, aplastic anemia, and liver fibrosis. Telomere shortening induces chromosomal instability that, in the absence of functional tumor suppressor genes, can contribute to tumorigenesis. In addition, mutations in telomere length maintenance genes and in shelterin components, the protein complex that protects telomeres, have been found to be associated with different types of cancer. These observations have encouraged the development of therapeutic strategies to treat and prevent telomere-associated diseases, namely aging-related diseases, including cancer. Here we review the molecular mechanisms underlying telomere-driven diseases and highlight recent advances in the preclinical development of telomere-targeted therapies using mouse models. PMID:28254828
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Barranco, R J; Gomez-Peralta, F; Abreu, C; Delgado-Rodriguez, M; Moreno-Carazo, A; Romero, F; de la Cal, M A; Barranco, J M; Pasquel, F J; Umpierrez, G E
2017-07-01
Hyperglycaemic crises (diabetic ketoacidosis and hyperosmolar hyperglycaemic state) are medical emergencies in people with diabetes. We aimed to determine their incidence, recurrence and economic impact. An observational study of hyperglycaemic crises cases using the database maintained by the out-of-hospital emergency service, the Healthcare Emergency Public Service (EPES) during 2012. The EPES provides emergency medical services to the total population of Andalusia, Spain (8.5 million inhabitants) and records data on the incidence, resource utilization and cost of out-of-hospital medical care. Direct costs were estimated using public prices for health services updated to 2012. Among 1 137 738 emergency calls requesting medical assistance, 3157 were diagnosed with hyperglycaemic crises by an emergency coordinator, representing 2.9 cases per 1000 persons with diabetes [95% confidence intervals (CI) 2.8 to 3.0]. The incidence of diabetic ketoacidosis was 2.5 cases per 1000 persons with diabetes (95% CI 2.4 to 2.6) and the incidence of hyperosmolar hyperglycaemic state was 0.4 cases per 1000 persons with diabetes (95% CI 0.4 to 0.5). In total, 17.7% (n = 440) of people had one or more hyperglycaemic crisis. The estimated total direct cost was €4 662 151, with a mean direct cost per episode of €1476.8 ± 217.8. Hyperglycaemic crises require high resource utilization of emergency medical services and have a significant economic impact on the health system. © 2017 Diabetes UK.
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Alvim, R C; Viana, M B; Pires, M A S; Franklin, H M O H; Paula, M J; Brito, A C; Oliveira, T F; Rezende, P V
2005-01-01
Analgesia and hydration remain the only safe treatment for painful crises of sickle cell disease; hydroxyurea is effective, but the toxicity is still a problem. Piracetam is a nootropic drug that has reportedly been effective and non-toxic in sickle cell patients, but most studies were not placebo-controlled and included a small number of patients. The present study evaluated the drug in a double-blind crossed placebo-controlled clinical trial in 73 children and adolescents suffering from moderate to severe painful crises for 13 months. Information regarding frequency and severity of pain was acquired through monthly clinical evaluation, visits and house calls, and 4,300 weekly questionnaires filled out by the patients in their domiciles. A monthly pain score was calculated for each patient. Pain was the most frequent adverse manifestation of the disease stressing its significant bio-psycho-social impact. Although nearly all patients and relatives reported a better clinical course throughout the whole study, the drug was ineffective in the prevention of painful crises. This placebo effect may be ascribed to an unplanned and unsystematic 'cognitive-behavioural' management of the children. The pain score in the second semester of the study - both in the experimental and in the control groups - was significantly smaller than that in the first semester. In conclusion, piracetam was found to be ineffective in the prevention of painful crises; a powerful placebo effect due to adequate patient care was demonstrated. Copyright (c) 2005 S. Karger AG, Basel
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Comparing emerging and mature markets during times of crises: A non-extensive statistical approach
NASA Astrophysics Data System (ADS)
Namaki, A.; Koohi Lai, Z.; Jafari, G. R.; Raei, R.; Tehrani, R.
2013-07-01
One of the important issues in finance and economics for both scholars and practitioners is to describe the behavior of markets, especially during times of crises. In this paper, we analyze the behavior of some mature and emerging markets with a Tsallis entropy framework that is a non-extensive statistical approach based on non-linear dynamics. During the past decade, this technique has been successfully applied to a considerable number of complex systems such as stock markets in order to describe the non-Gaussian behavior of these systems. In this approach, there is a parameter q, which is a measure of deviation from Gaussianity, that has proved to be a good index for detecting crises. We investigate the behavior of this parameter in different time scales for the market indices. It could be seen that the specified pattern for q differs for mature markets with regard to emerging markets. The findings show the robustness of the stated approach in order to follow the market conditions over time. It is obvious that, in times of crises, q is much greater than in other times. In addition, the response of emerging markets to global events is delayed compared to that of mature markets, and tends to a Gaussian profile on increasing the scale. This approach could be very useful in application to risk and portfolio management in order to detect crises by following the parameter q in different time scales.
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Ryan, Grace Kathryn; Bauer, Andreas; Bass, Judith K; Eaton, Julian
2018-02-17
There is a severe shortage of specialist mental healthcare providers in low-income and middle-income countries (LMICs) affected by humanitarian crises. In these settings, talking therapies may be delivered by non-specialists, including lay workers with no tertiary education or formal certification in mental health. This systematic review will synthesise the literature on the implementation and effectiveness of talking therapies delivered by lay workers in LMICs affected by humanitarian crises, in order to develop a Theory of Change (ToC). Qualitative, quantitative and mixed-methods studies assessing the implementation or effectiveness of lay-delivered talking therapies for common mental disorders provided to adult survivors of humanitarian crises in LMICs will be eligible for inclusion. Studies set in high-income countries will be excluded. No restrictions will be applied to language or year of publication. Unpublished studies will be excluded. Seven electronic databases will be searched: MEDLINE, Embase, PsycINFO, PsycEXTRA, Global Health, Cochrane Library and ClinicalTrials.gov. Contents pages of three peer-reviewed journals will be hand-searched. Sources of grey literature will include resource directories of two online mental health networks (MHPSS.net and MHInnovation.net) and expert consultation. Forward and backward citation searches of included studies will be performed. Two reviewers will independently screen studies for inclusion, extract data and assess study quality. A narrative synthesis will be conducted, following established guidelines. A ToC map will be amended iteratively to take into account the review results and guide the synthesis. Findings will be presented in a manuscript for publication in a peer-reviewed journal and disseminated through a coordinated communications strategy targeting knowledge generators, enablers and users. CRD42017058287. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
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Ryan, Grace Kathryn; Bass, Judith K; Eaton, Julian
2018-01-01
Introduction There is a severe shortage of specialist mental healthcare providers in low-income and middle-income countries (LMICs) affected by humanitarian crises. In these settings, talking therapies may be delivered by non-specialists, including lay workers with no tertiary education or formal certification in mental health. This systematic review will synthesise the literature on the implementation and effectiveness of talking therapies delivered by lay workers in LMICs affected by humanitarian crises, in order to develop a Theory of Change (ToC). Methods and analysis Qualitative, quantitative and mixed-methods studies assessing the implementation or effectiveness of lay-delivered talking therapies for common mental disorders provided to adult survivors of humanitarian crises in LMICs will be eligible for inclusion. Studies set in high-income countries will be excluded. No restrictions will be applied to language or year of publication. Unpublished studies will be excluded. Seven electronic databases will be searched: MEDLINE, Embase, PsycINFO, PsycEXTRA, Global Health, Cochrane Library and ClinicalTrials.gov. Contents pages of three peer-reviewed journals will be hand-searched. Sources of grey literature will include resource directories of two online mental health networks (MHPSS.net and MHInnovation.net) and expert consultation. Forward and backward citation searches of included studies will be performed. Two reviewers will independently screen studies for inclusion, extract data and assess study quality. A narrative synthesis will be conducted, following established guidelines. A ToC map will be amended iteratively to take into account the review results and guide the synthesis. Ethics and dissemination Findings will be presented in a manuscript for publication in a peer-reviewed journal and disseminated through a coordinated communications strategy targeting knowledge generators, enablers and users. PROSPERO registration number CRD42017058287. PMID:29455163
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Planetary Bootstrap: A Prelude to Biosphere Phenomenology
NASA Astrophysics Data System (ADS)
Kazansky, Alexander B.
2004-08-01
This paper deals with systemic status as well as with some phenomenological and evolutionary aspects of biosphere. Biosphere is represented as multilevel autopoietic system in which different organizational levels are nested into each other. The conceptual model of punctuated epigenesis, biosphere evolutionary process is suggested, in which endogenous planetary organizational crises play role of evolutionary mechanism, creating novelty. The hypothesis is proposed, that the biosphere reaction on the humankind destructive activity reminds the distributed immune response of biological organism, described by F.Varela in his "cognitive immunology". The biosphere evolution is interpreted as the hermeneutical spiral of "Process Being" self-uncovering thus illustrating the historical process of transformation of biosphere as the type of Being in the periods of crises. Some arguments are adduced in favor of biosphere phenomenology development and application of the methods of second-order cybernetics to actual problems of planetary scale.
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Tapia Rico, Gonzalo; Li, Minmin; Pavlakis, Nick; Cehic, Gabrielle; Price, Timothy J
2018-05-01
Peptide receptor radionuclide therapy (PRRT) is an important therapeutic option for somatostatin receptor (SSTR) positive metastatic and/or inoperable neuroendocrine tumours (NETs). However, in patients with poorly controlled carcinoid syndrome, it may lead to an acute flare of carcinoid symptoms or even carcinoid crisis. We report seven patients who received PRRT with ( 177 Lu-DOTA 0 , Tyr 3 ) octreotate ( 177 Lu-octreotate-LuTate) across two Australian tertiary medical institutions who developed acute flare of carcinoid symptoms/carcinoid crisis during/after PRRT. Cases were identified as high-risk due to previous history of carcinoid crises, high tumour burden and markedly elevated tumour markers. We propose a protocol to prevent and manage severe carcinoid symptoms in high-risk patients treated with PRRT. Copyright © 2018 Elsevier Ltd. All rights reserved.
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Economic crisis and mental health.
Uutela, Antti
2010-03-01
Literature from the past year was examined to learn whether economic recessions have an effect on mental disorders including depression and suicides. Economic recessions and crises have a context-dependent negative impact on mental health disorders. These appear in low-income and middle-income countries whereas some affluent countries are offering provisions that help unemployed persons to escape the detrimental consequences. The Asian economic crisis led to a sharp unemployment-related increase in suicide mortality in east Asian countries. In European Union countries rising unemployment was associated with significant short-term increases in premature deaths from intentional violence including suicides. It seems that active labour market programmes existing in some Organization for Economic Cooperation and Development countries can prevent some adverse health effects of economic downturns. As mental health consequences of economic crises are context dependent, the current situation needs monitoring. Enough services for those in need should be provided and advocacy for societal support measures is of great importance.
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Fredrickson, Barbara L.; Tugade, Michele M.; Waugh, Christian E.; Larkin, Gregory R.
2009-01-01
Extrapolating from B. L. Fredrickson's (1998, 2001) broaden-and-build theory of positive emotions, the authors hypothesized that positive emotions are active ingredients within trait resilience. U.S. college students (18 men and 28 women) were tested in early 2001 and again in the weeks following the September 11th terrorist attacks. Mediational analyses showed that positive emotions experienced in the wake of the attacks— gratitude, interest, love, and so forth—fully accounted for the relations between (a) precrisis resilience and later development of depressive symptoms and (b) precrisis resilience and postcrisis growth in psychological resources. Findings suggest that positive emotions in the aftermath of crises buffer resilient people against depression and fuel thriving, consistent with the broaden-and-build theory. Discussion touches on implications for coping. PMID:12585810
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Ramesh, Anita; Blanchet, Karl; Ensink, Jeroen H J; Roberts, Bayard
2015-01-01
Water, sanitation, and hygiene (WASH) interventions are amongst the most crucial in humanitarian crises, although the impact of the different WASH interventions on health outcomes remains unclear. To examine the quantity and quality of evidence on WASH interventions on health outcomes in humanitarian crises, as well as evaluate current evidence on their effectiveness against health outcomes in these contexts. A systematic literature review was conducted of primary and grey quantitative literature on WASH interventions measured against health outcomes in humanitarian crises occurring from 1980-2014. Populations of interest were those in resident in humanitarian settings, with a focus on acute crisis and early recovery stages of humanitarian crises in low and middle-income countries. Interventions of interest were WASH-related, while outcomes of interest were health-related. Study quality was assessed via STROBE/CONSORT criteria. Results were analyzed descriptively, and PRISMA reporting was followed. Of 3963 studies initially retrieved, only 6 published studies measured a statistically significant change in health outcome as a result of a WASH intervention. All 6 studies employed point-of-use (POU) water quality interventions, with 50% using safe water storage (SWS) and 35% using household water treatment (HWT). All 6 studies used self-reported diarrhea outcomes, 2 studies also reported laboratory confirmed outcomes, and 2 studies reported health treatment outcomes (e.g. clinical admissions). 1 study measured WASH intervention success in relation to both health and water quality outcomes; 1 study recorded uptake (use of soap) as well as health outcomes. 2 studies were unblinded randomized-controlled trials, while 4 were uncontrolled longitudinal studies. 2 studies were graded as providing high quality evidence; 3 studies provided moderate and 1 study low quality evidence. The current evidence base on the impact of WASH interventions on health outcomes in humanitarian crises is extremely limited, and numerous methodological limitations limit the ability to determine associative, let alone causal, relationships.
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Ramesh, Anita; Blanchet, Karl; Ensink, Jeroen H. J.; Roberts, Bayard
2015-01-01
Background Water, sanitation, and hygiene (WASH) interventions are amongst the most crucial in humanitarian crises, although the impact of the different WASH interventions on health outcomes remains unclear. Aim To examine the quantity and quality of evidence on WASH interventions on health outcomes in humanitarian crises, as well as evaluate current evidence on their effectiveness against health outcomes in these contexts. Methods A systematic literature review was conducted of primary and grey quantitative literature on WASH interventions measured against health outcomes in humanitarian crises occurring from 1980–2014. Populations of interest were those in resident in humanitarian settings, with a focus on acute crisis and early recovery stages of humanitarian crises in low and middle-income countries. Interventions of interest were WASH-related, while outcomes of interest were health-related. Study quality was assessed via STROBE/CONSORT criteria. Results were analyzed descriptively, and PRISMA reporting was followed. Results Of 3963 studies initially retrieved, only 6 published studies measured a statistically significant change in health outcome as a result of a WASH intervention. All 6 studies employed point-of-use (POU) water quality interventions, with 50% using safe water storage (SWS) and 35% using household water treatment (HWT). All 6 studies used self-reported diarrhea outcomes, 2 studies also reported laboratory confirmed outcomes, and 2 studies reported health treatment outcomes (e.g. clinical admissions). 1 study measured WASH intervention success in relation to both health and water quality outcomes; 1 study recorded uptake (use of soap) as well as health outcomes. 2 studies were unblinded randomized-controlled trials, while 4 were uncontrolled longitudinal studies. 2 studies were graded as providing high quality evidence; 3 studies provided moderate and 1 study low quality evidence. Conclusion The current evidence base on the impact of WASH interventions on health outcomes in humanitarian crises is extremely limited, and numerous methodological limitations limit the ability to determine associative, let alone causal, relationships. PMID:26398228
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2018-03-05
Acute Myeloid Leukemia in Remission; Adult Acute Lymphoblastic Leukemia in Complete Remission; Chronic Myelogenous Leukemia, BCR-ABL1 Positive in Remission; Chronic Myelomonocytic Leukemia in Remission; Graft Versus Host Disease; Hodgkin Lymphoma; Minimal Residual Disease; Myelodysplastic Syndrome; Myeloproliferative Neoplasm; Non-Hodgkin Lymphoma; Plasma Cell Myeloma; Severe Aplastic Anemia; Waldenstrom Macroglobulinemia
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A Multicenter Access and Distribution Protocol for Unlicensed Cryopreserved Cord Blood Units (CBUs)
2018-05-15
Hematologic Malignancies; Inherited Disorders of Metabolism; Inherited Abnormalities of Platelets; Histiocytic Disorders; Acute Myelogenous Leukemia (AML or ANLL); Acute Lymphoblastic Leukemia (ALL); Other Acute Leukemia; Chronic Myelogenous Leukemia (CML); Myelodysplastic (MDS) / Myeloproliferative (MPN) Diseases; Other Leukemia; Hodgkin Lymphoma; Non-hodgkin Lymphoma; Multiple Myeloma/ Plasma Cell Disorder (PCD); Inherited Abnormalities of Erythrocyte Differentiation or Function; Disorders of the Immune System; Automimmune Diseases; Severe Aplastic Anemia
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Piracetam for reducing the incidence of painful sickle cell disease crises.
Al Hajeri, A A; Fedorowicz, Z; Omran, A; Tadmouri, G O
2007-04-18
Sickle cell disease is one of the most common genetic disorders. Sickle cell crises in which irregular and dehydrated cells contribute to blocking of blood vessels are characterised by episodes of pain. Treatment is mainly supportive and symptomatic. In vitro studies with piracetam indicate that it has the potential for inhibition and a reversal of the process of sickling of erythrocytes. To assess the effectiveness of piracetam for reducing the incidence of painful sickle cell disease crises. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of the last search of the Group's Haemoglobinopathies Trials Register: February 2007. Randomised controlled trials comparing orally administered piracetam to placebo or standard care in people, of all ages and both sexes, with sickle cell disease. Two authors independently assessed trial quality and extracted data. Trial authors were contacted for additional information. Adverse effects data were collected from the trials. Three trials involving 169 participants were included in the review. A limited amount of data addressing some of the primary and some of the secondary outcomes were provided, but data were incomplete and based on unvalidated assumptions used in the evaluation of outcomes. One trial reported a reduction in the number of pain crises and their severity with active intervention than placebo but presented no data to confirm these results. A second trial presented a monthly global pain score based on the number of sickle cell crises and severity of pain but included no separate data for these primary outcomes. Although there was no significant difference between the piracetam and placebo periods for the number of days of hospitalisation (P = 0.87) in one trial, inconsistencies in the criteria necessary for hospitalisation during sickle crises did not permit accurate conclusions to be drawn. Two of the trials reported participant satisfaction with piracetam but provided no details as to how this satisfaction had been assessed. There were no reports of toxicity or adverse effects with piracetam other than one participant who experienced dizziness. The small number of included trials and their poor methodological quality provided insufficient reliable evidence to support the routine use of this medication for preventing the incidence of painful sickle cell disease crises.
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Aldehyde dehydrogenase 2 in aplastic anemia, Fanconi anemia and hematopoietic stem cells.
Van Wassenhove, Lauren D; Mochly-Rosen, Daria; Weinberg, Kenneth I
2016-09-01
Maintenance of the hematopoietic stem cell (HSC) compartment depends on the ability to metabolize exogenously and endogenously generated toxins, and to repair cellular damage caused by such toxins. Reactive aldehydes have been demonstrated to cause specific genotoxic injury, namely DNA interstrand cross-links. Aldehyde dehydrogenase 2 (ALDH2) is a member of a 19 isoenzyme ALDH family with different substrate specificities, subcellular localization, and patterns of expression. ALDH2 is localized in mitochondria and is essential for the metabolism of acetaldehyde, thereby placing it directly downstream of ethanol metabolism. Deficiency in ALDH2 expression and function are caused by a single nucleotide substitution and resulting amino acid change, called ALDH2*2. This genetic polymorphism affects 35-45% of East Asians (about ~560 million people), and causes the well-known Asian flushing syndrome, which results in disulfiram-like reactions after ethanol consumption. Recently, the ALDH2*2 genotype has been found to be associated with marrow failure, with both an increased risk of sporadic aplastic anemia and more rapid progression of Fanconi anemia. This review discusses the unexpected interrelationship between aldehydes, ALDH2 and hematopoietic stem cell biology, and in particular its relationship to Fanconi anemia. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.
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Babushok, Daria V.; Bessler, Monica; Olson, Timothy S.
2016-01-01
Myelodysplastic syndrome (MDS) is a clonal blood disorder characterized by ineffective hematopoiesis, cytopenias, dysplasia and an increased risk of acute myeloid leukemia (AML). With the growing availability of clinical genetic testing, there is an increasing appreciation that a number of genetic predisposition syndromes may underlie apparent de novo presentations of MDS/AML, particularly in children and young adults. Recent findings of clonal hematopoiesis in acquired aplastic anemia add another facet to our understanding of the mechanisms of MDS/AML predisposition. As more predisposition syndromes are recognized, it is becoming increasingly important for hematologists and oncologists to have familiarity with the common as well as emerging syndromes, and to have a systematic approach to diagnosis and screening of at risk patient populations. Here, we provide a practical algorithm for approaching a patient with a suspected MDS/AML predisposition, and provide an in-depth review of the established and emerging familial MDS/AML syndromes caused by mutations in the ANKRD26, CEBPA, DDX41, ETV6, GATA2, RUNX1, SRP72 genes. Finally, we discuss recent data on the role of somatic mutations in malignant transformation in acquired aplastic anemia, and review the practical aspects of MDS/AML management in patients and families with predisposition syndromes. PMID:26693794
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Clinical presentations of parvovirus B19 infection.
Servey, Jessica T; Reamy, Brian V; Hodge, Joshua
2007-02-01
Although most persons with parvovirus B19 infection are asymptomatic or have mild, nonspecific, cold-like symptoms, several clinical conditions have been linked to the virus. Parvovirus B19 usually infects children and causes the classic "slapped-cheek" rash of erythema infectiosum (fifth disease). The virus is highly infectious and spreads mainly through respiratory droplets. By the time the rash appears, the virus is no longer infectious. The virus also may cause acute or persistent arthropathy and papular, purpuric eruptions on the hands and feet ("gloves and socks" syndrome) in adults. Parvovirus B19 infection can trigger an acute cessation of red blood cell production, causing transient aplastic crisis, chronic red cell aplasia, hydrops fetalis, or congenital anemia. This is even more likely in patients with illnesses that have already shortened the lifespan of erythrocytes (e.g., iron deficiency anemia, human immunodeficiency virus, sickle cell disease, thalassemia, spherocytosis). A clinical diagnosis can be made without laboratory confirmation if erythema infectiosum is present. If laboratory confirmation is needed, serum immunoglobulin M testing is recommended for immunocompetent patients; viral DNA testing is recommended for patients in aplastic crisis and for those who are immunocompromised. Treatment is usually supportive, although some patients may require transfusions or intravenous immune globulin therapy. Most patients recover completely.
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The common causes leading to pancytopenia in patients presenting to tertiary care hospital.
Das Makheja, Kirpal; Kumar Maheshwari, Bharat; Arain, Shafique; Kumar, Suneel; Kumari, Sangeeta; Vikash
2013-09-01
The objective of this study was to determine the frequency of common causes leading to Pancytopenia in patients presenting to tertiary care hospital at Karachi. A total of 62 patients with the diagnosis of Pancytopenia of more than one week duration were enrolled in the study. All patients underwent a detailed medical history and full physical examination followed by blood sampling for the investigations i.e. complete blood count with peripheral film, erythrocyte sedimentation rate (ESR), Malarial parasites (MP), liver function test, Renal function tests, PT and viral profile (HBsAg, Anti-HCV), Ultrasonography of abdomen. All patients underwent bone marrow aspiration and trephine biopsy for reporting and interpretation. Duration of study was six months, from May 2010 to November 2010. The average age of the patients was 37.76 ± 16.38years. Out of 62 patients, 36 (58%) were male and 26 (42%) were female. Megaloblastic anemia was the commonest cause that was observed in 41.9% cases followed by acute myeloid leukemia 27.4%, aplastic anemia 19.4% and erythroid hyperplasia 11.3%. Conclusion : This study concluded that most common cause of pancytopenia is Megaloblastic anemia, followed by acute myeloid leukemia and aplastic anemia. Bone marrow examination is a single useful investigation which reveals the underlying cause in patients with pancytopenia.
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Maciejewski, Jaroslaw P; Balasubramanian, Suresh K
2017-12-08
Recent technological advances in genomics have led to the discovery of new somatic mutations and have brought deeper insights into clonal diversity. This discovery has changed not only the understanding of disease mechanisms but also the diagnostics and clinical management of bone marrow failure. The clinical applications of genomics include enhancement of current prognostic schemas, prediction of sensitivity or refractoriness to treatments, and conceptualization and selective application of targeted therapies. However, beyond these traditional clinical aspects, complex hierarchical clonal architecture has been uncovered and linked to the current concepts of leukemogenesis and stem cell biology. Detection of clonal mutations, otherwise typical of myelodysplastic syndrome, in the course of aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria has led to new pathogenic concepts in these conditions and created a new link between AA and its clonal complications, such as post-AA and paroxysmal nocturnal hemoglobinuria. Distinctions among founder vs subclonal mutations, types of clonal evolution (linear or branching), and biological features of individual mutations (sweeping, persistent, or vanishing) will allow for better predictions of the biologic impact they impart in individual cases. As clonal markers, mutations can be used for monitoring clonal dynamics of the stem cell compartment during physiologic aging, disease processes, and leukemic evolution. © 2016 by The American Society of Hematology. All rights reserved.
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Parvovirus B19 Associated Hepatitis
Bihari, Chhagan; Rastogi, Archana; Saxena, Priyanka; Rangegowda, Devraj; Chowdhury, Ashok; Gupta, Nalini; Sarin, Shiv Kumar
2013-01-01
Parvovirus B19 infection can present with myriads of clinical diseases and syndromes; liver manifestations and hepatitis are examples of them. Parvovirus B19 hepatitis associated aplastic anemia and its coinfection with other hepatotropic viruses are relatively underrecognized, and there is sufficient evidence in the literature suggesting that B19 infections can cause a spectrum of liver diseases from elevation of transaminases to acute hepatitis to fulminant liver failure and even chronic hepatitis. It can also cause fatal macrophage activation syndrome and fibrosing cholestatic hepatitis. Parvovirus B19 is an erythrovirus that can only be replicate in pronormoblasts and hepatocytes, and other cells which have globosides and glycosphingolipids in their membrane can also be affected by direct virus injury due to nonstructural protein 1 persistence and indirectly by immune mediated injury. The virus infection is suspected in bone marrow aspiration in cases with sudden drop of hemoglobin and onset of transient aplastic anemia in immunosuppressed or immunocompetent patients and is confirmed either by IgM and IgG positive serology, PCR analysis, and in situ hybridization in biopsy specimens or by application of both. There is no specific treatment for parvovirus B19 related liver diseases, but triple therapy regimen may be effective consisting of immunoglobulin, dehydrohydrocortisone, and cyclosporine. PMID:24232179
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Lens, Sabela; Calleja, Jose L; Campillo, Ana; Carrión, Jose A; Broquetas, Teresa; Perello, Christie; de la Revilla, Juan; Mariño, Zoe; Londoño, María-Carlota; Sánchez-Tapias, Jose M; Urbano-Ispizua, Álvaro; Forns, Xavier
2015-01-01
Telaprevir and Boceprevir are the first direct acting antivirals approved for chronic hepatitis C in combination with peg-interferon alfa and ribavirin. Pancytopenia due to myelotoxicity caused by these drugs may occur, but severe hematological abnormalities or aplastic anemia (AA) have not been described. We collected all cases of severe pancytopenia observed during triple therapy with telaprevir in four Spanish centers since approval of the drug in 2011. Among 142 cirrhotic patients receiving treatment, 7 cases of severe pancytopenia (5%) were identified and three were consistent with the diagnosis of AA. Mean age was 59 years, five patients had compensated cirrhosis and two patients had severe hepatitis C recurrence after liver transplantation. Severe pancytopenia was diagnosed a median of 10 wk after the initiation of therapy. Three patients had pre-treatment hematological abnormalities related to splenomegaly. In six patients, antiviral treatment was interrupted at the onset of hematological abnormalities. Two patients died due to septic complications and one patient due to acute alveolar hemorrhage. The remaining patients recovered. Severe pancytopenia and especially AA, are not rare during triple therapy with telaprevir in patients with advanced liver disease. Close monitoring is imperative in this setting to promptly detect serious hematological disorders and to prevent further complications. PMID:25954117
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Evolving Hematopoietic Stem Cell Transplantation Strategies in Severe Aplastic Anemia
Dietz, Andrew C.; Lucchini, Giovanna; Samarasinghe, Sujith; Pulsipher, Michael A.
2016-01-01
Purpose of Review Significant improvements in unrelated donor hematopoietic stem cell transplantation (HSCT) in recent years has solidified its therapeutic role in severe aplastic anemia (SAA) and led to evolution of treatment algorithms, particularly for children. Recent Findings Advances in understanding genetics of inherited bone marrow failure syndromes (IBMFS) have allowed more confidence in accurately diagnosing SAA and avoiding treatments that could be dangerous and ineffective in individuals with IBMFS, which can be diagnosed in 10–20% of children presenting with a picture of SAA. Additionally long-term survival after matched sibling donor (MSD) and matched unrelated donor (MUD) HSCT now exceed 90% in children. Late effects after HSCT for SAA are minimal with current strategies and compare favorably to late effects after up-front immunosuppressive therapy (IST), except for patients with chronic graft versus host disease (GVHD). Summary 1) Careful assessment for signs or symptoms of IBMFS along with genetic screening for these disorders is of major importance. 2) MSD HSCT is already considered standard of care for up-front therapy and some groups are evaluating MUD HSCT as primary therapy. 3) Ongoing studies will continue to challenge treatment algorithms and may lead to an even more expanded role for HSCT in SAA. PMID:26626557
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Long-term outcome of pregnancy complicating with severe aplastic anemia under supportive care.
Chen, Kuan-Ju; Chang, Yao-Lung; Chang, Horng; Su, Shen-Yuan; Peng, Hsiu-Huei; Chang, Shuenn-Dyh; Chao, An-Shine
2017-10-01
Pregnancy associated with aplastic anemia (AA) is a rare and heterogeneous disorder. We aimed to identify and evaluate the maternal and pregnant outcomes of pregnancy-associated severe AA treated with supportive care. A 25-year retrospective study was conducted at in a single center between 1990 and 2014 with pregnancy associated severe AA. In addition, relevant published cases of antenatally diagnosed pregnancy-associated severe AA after 1990 were identified by PubMed. The main goal was to determine the impact of various risk factors on maternal and fetal outcomes. 15 women with 18 pregnancies were enrolled. With addition of the published reports in literature, a total of 36 cases were included for reference review. Univariate analysis showed that low platelet counts (<2.0 × 10 9 /L), bone marrow hypocellularity (<25%), and late diagnosis during pregnancy were predictors of poor maternal outcomes (P < 0.05). The complication rate of pregnancy outcomes was 53.3%, including preterm delivery, small gestational age (SGA), preterm premature ruptured of membranes (PPROM) and preeclampsia. This study identified the risk factors of mortality and morbidity in pregnant women with severe AA, as well as the obstetrical complications associated with neonatal outcome. Copyright © 2017. Published by Elsevier B.V.
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Wu, Jun; Cheng, Yifei; Zhang, Leping
2015-12-01
This retrospective single-center study assessed the incidence and clinical features of immune manifestations of refractory cytopenia of childhood (RCC) and childhood aplastic anemia (AA). We evaluated 72 children with RCC and 123 with AA between February 2008 and March 2013. RCC was associated with autoimmune disease in 4 children, including 1 case each with autoimmune hemolytic anemia, rheumatoid arthritis, systemic lupus erythematosus, and anaphylactoid purpura. No children with AA were diagnosed with autoimmune diseases. Immune abnormalities were common in both RCC and AA; the most significant reductions were in the relative numbers of CD3-CD56+ subsets found in RCC. Despite the many similar immunologic abnormalities in AA and RCC, the rate of autoimmune disease was significantly lower in childhood AA than RCC (p=0.008, χ2=6.976). The relative numbers of natural killer cells were significantly lower in RCC patients than AA patients. By month 6, there was no significant difference in autoimmune manifestations between RCC and AA in relation to the response to immunosuppressive therapy (p=0.907, χ2=0.014). The large overlap of analogous immunologic abnormalities indicates that RCC and childhood AA may share the same pathogenesis. Copyright © 2015 Elsevier Ltd. All rights reserved.
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Zhu, Hua; Luo, Rong Mu; Luan, Zuo; Lee, Vincent; Zhu, Yi Ping; Luo, Cheng Juan; Tang, Xiang Feng; Si, Ying Jian; Chen, Jing
2016-09-01
Haploidentical haematopoietic stem cell transplantation (haplo-HSCT) used to be a third-line treatment option for childhood severe aplastic anaemia (SAA). We conducted this retrospective study of 36 children (38 transplants) who received haplo-HSCT from human leucocyte antigen (HLA)-mismatched related donors between July 2002 and November 2013 at five HSCT centres in China, including 17 cases that were 5/6 HLA matched (Group 1) and 21 that were 4/6 or 3/6 HLA matched (Group 2). Although patients in Group 2 had a higher incidence of grade II-IV acute graft-versus-host disease (57·9% vs. 5·9%, P = 0·001), they had similar rates of graft failure (5·3% vs. 5·9%, P = 0·742) and overall survival (80·8% vs. 93·8%, P = 0·234) as Group 1. Unmanipulated haplo-HSCT is an effective treatment for SAA children with satisfactory outcome of this cohort, especially in the 5/6 HLA-matched group. For patients in critical situations, such as unresponsive to immunosuppressive therapy, refractory infection and failing first HSCT, to bring forward the timing of haplo-HSCT is a feasible salvage strategy with better and faster donor accessibility. © 2016 John Wiley & Sons Ltd.
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Hematopoietic stem cell transplantation for acquired aplastic anemia
Georges, George E.; Storb, Rainer
2016-01-01
Purpose of review There has been steady improvement in outcomes with allogeneic bone marrow transplantation (BMT) for severe aplastic anemia (SAA), due to progress in optimization of the conditioning regimens, donor hematopoietic cell source and supportive care. Here we review recently published data that highlight the improvements and current issues in the treatment of SAA. Recent findings Approximately one-third of AA patients treated with immune suppression therapy (IST) have acquired mutations in myeloid cancer candidate genes. Because of the greater probability for eventual failure of IST, human leukocyte antigen (HLA)-matched sibling donor BMT is the first-line of treatment for SAA. HLA-matched unrelated donor (URD) BMT is generally recommended for patients who have failed IST. However, in younger patients for whom a 10/10-HLA-allele matched URD can be rapidly identified, there is a strong rationale to proceed with URD BMT as first-line therapy. HLA-haploidentical BMT using post-transplant cyclophosphamide (PT-CY) conditioning regimens, is now a reasonable second-line treatment for patients who failed IST. Summary Improved outcomes have led to an increased first-line role of BMT for treatment of SAA. The optimal cell source from an HLA-matched donor is bone marrow. Additional studies are needed to determine the optimal conditioning regimen for HLA-haploidentical donors. PMID:27607445
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Short telomeres: from dyskeratosis congenita to sporadic aplastic anemia and malignancy.
Gramatges, Maria M; Bertuch, Alison A
2013-12-01
Telomeres are DNA-protein structures that form a protective cap on chromosome ends. As such, they prevent the natural ends of linear chromosomes from being subjected to DNA repair activities that would result in telomere fusion, degradation, or recombination. Both the DNA and protein components of the telomere are required for this essential function, because insufficient telomeric DNA length, loss of the terminal telomeric DNA structure, or deficiency of key telomere-associated factors may elicit a DNA damage response and result in cellular senescence or apoptosis. In the setting of failed checkpoint mechanisms, such DNA-protein defects can also lead to genomic instability through telomere fusions or recombination. Thus, as shown in both model systems and in humans, defects in telomere biology are implicated in cellular and organismal aging as well as in tumorigenesis. Bone marrow failure and malignancy are 2 life-threatening disease manifestations in the inherited telomere biology disorder dyskeratosis congenita. We provide an overview of basic telomere structure and maintenance. We outline the telomere biology defects observed in dyskeratosis congenita, focusing on recent discoveries in this field. Last, we review the evidence of how telomere biology may impact sporadic aplastic anemia and the risk for various cancers. Copyright © 2013 Mosby, Inc. All rights reserved.
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Charting the Impacts of University-Child Welfare Collaboration.
ERIC Educational Resources Information Center
Briar-Lawson, Katharine, Ed.; Zlotnik, Joan Levy, Ed.
Few fields of service command as much public attention as child welfare. Workforce development is often a less visible yet critical response to service delivery crises and efforts to improve child and family outcomes. In the late 1980s, a campaign was undertaken to rebuild public child welfare systems with trained social workers. The subsequent…
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A Crisis in Space--A Futuristic Simulation Using Creative Problem Solving.
ERIC Educational Resources Information Center
Clode, Linda
1992-01-01
An enrichment program developed for sixth-grade gifted students combined creative problem solving with future studies in a way that would simulate real life crisis problem solving. The program involved forecasting problems of the future requiring evacuation of Earth, assuming roles on a spaceship, and simulating crises as the spaceship traveled to…
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Same-Page Lessons from Front-Page Crises
ERIC Educational Resources Information Center
Simpson, Christopher
2006-01-01
In a university's time of crisis, reporters will often attempt to catch the president and board members in conflicting responses in order to develop a "gotcha" news story. It is important for leaders not to take the bait. In times of crisis, board members and institutional leaders must not only control the message and flow of…
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Dynamic Changes of Striatal and Extrastriatal Abnormalities in Glutaric Aciduria Type I
ERIC Educational Resources Information Center
Harting, Inga; Neumaier-Probst, Eva; Seitz, Angelika; Maier, Esther M.; Assmann, Birgit; Baric, Ivo; Troncoso, Monica; Muhlhausen, Chris; Zschocke, Johannes; Boy, Nikolas P. S.; Hoffmann, Georg F.; Garbade, Sven F.; Kolker, Stefan
2009-01-01
In glutaric aciduria type I, an autosomal recessive disease of mitochondrial lysine, hydroxylysine and tryptophan catabolism, striatal lesions are characteristically induced by acute encephalopathic crises during a finite period of brain development (age 3-36 months). The frequency of striatal injury is significantly less in patients diagnosed as…
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Effective Crisis Management at the Smaller Campus.
ERIC Educational Resources Information Center
DeWitt, Robert C.
Pennsylvania State University Beaver Campus developed crisis guidelines and a formal working relationship with a local community mental health center in order to be able to deal with on-campus crises and their followup. The guidelines provide each employee with a single, easy-to-follow document that outlines the decision making process to be…
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Learning: Meeting the Challenges of Older Adulthood.
ERIC Educational Resources Information Center
Wolf, Mary Alice
Erik Erikson's model (1963, 1982) is most useful to an understanding of development and aging. He describes lifelong growth as related to tasks that must be performed. At each stage of life, times of stability are followed by developmental crises. Upon resolving the crisis, the individual can enjoy the particular beauty and security of that…
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Meeting the Challenges of ESD Competency-Based Curriculum in a Vocational School Setting
ERIC Educational Resources Information Center
Iliško, Dzintra; Olehnovica, Eridiana; Ostrovska, Inta; Akmene, Velga; Salite, Ilga
2017-01-01
Sustainability is becoming an integral part of vocational schools since schools are called to respond to the environmental crises and unsustainability issues in the community as well as to an unsustainable economic development. Vocational schools have to play a significant role is re-orienting students' frames of reference towards sustainability…
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Do It Now: Short-Term Responses to Traumatic Events
ERIC Educational Resources Information Center
Demaria, Thomas; Schonfeld, David J.
2014-01-01
Traumatic crises can have a lifelong effect. The majority of children, however, will not develop lasting problems following a crisis. The immediate goal of the school is to minimize the negative impact, support coping, accelerate adjustment, and instill confidence in students that school staff is available to address their needs. Support and…
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What Are the Attributes and Duties of the School Crisis Intervention Team?
ERIC Educational Resources Information Center
Gullatt, David E.; Long, Douglas
1996-01-01
Physical measures such as weapons checks and metal detectors are inadequate to forestall school violence. The key to managing crises is a trained, broad-based crisis-intervention team and a crisis-management plan. Team responsibilities include developing an intervention plan, coordinating with community services, educating and training staff, and…
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Resources in Crisis Intervention: School, Family and Community Applications.
ERIC Educational Resources Information Center
Sandoval, Jonathan, Ed.
Originally developed to respond to the Desert Storm/Persian Gulf War, this resource book provides information designed to help parents, teachers, and service providers to plan and implement a comprehensive response to meeting children's emotional needs during a crisis. Contributors are experts on different kinds of crises that school age children…
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Can Events Predict Violent Intra-State Crises?
2011-01-01
violences politiques et à attirer des organisations terroristes, à qui ils fournissent un refuge sûr. Ces situations concernent le Canada, tout...Number of Women Parliamentarians Inequality Score (GINI Coefficient) Percentage of Women Parliamentarians Unemployment by sex (Female...Military Expenditure (% of GDP) Political violence of civilians (fatalities) Human Development (H) Organised crime Child malnourishment
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Community College Presidents' Decision-Making Processes during a Potential Crisis
ERIC Educational Resources Information Center
Berry, Judith Kaye
2013-01-01
This case study addressed how community college presidents make decisions under conditions that can escalate to full-scale crises. The purpose of this study was to gather data to support the development of alternative models or refinement of existing models for crisis decision making on community college campuses, using an abbreviated…
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Materials Technology; 200 Years and the Future.
ERIC Educational Resources Information Center
Yadon, James N.; Steeb, Ralph V.
Focus in this paper is on the importance of materials technology, the matter and energy crises, and the interrelatedness of our increasing need for materials, and the implications for education. Following a short history of what materials have done for man and what man has done with materials, particularly in the development of various metals and…
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1986-03-01
SCHIZOPHRENIC DISORDER Amphetamine Delusional Disorder PARANOID DISORDER Cannabis Delusional Disorder Hallucinogen Delusional Disorder BRIEF REACTIVE...Intoxication Borderline Personality Disorder Cocaine Intoxication Histrionic Personality Disorder Caffeine Intoxication Narcissistic Personality Disorder... Cannabis Intoxication Dependent Personality Disorder ON, Inhalant Intoxication Schizoid Personality Disorder Minor Tranquilizer Intoxication Compulsive
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The Crisis Manual for Early Childhood Teachers: How To Handle the Really Difficult Problems.
ERIC Educational Resources Information Center
Miller, Karen
More and more teachers report the increasing incidence of crises in children's lives, crises that interfere with the child's ability to learn. Noting that developmentally appropriate curricula must respond to the issues of immediate concern and interest to children, this source book assists teachers in facing difficult issues in the classroom and…
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ERIC Educational Resources Information Center
US Department of Education, 2008
2008-01-01
"Lessons Learned" is a series of publications that are a brief recounting of actual school emergencies and crises. This "Lessons Learned" issue examines the incidence of student walkout demonstrations and the various ways in which administrators, school staff, law enforcement, and the community at large can help keep youths…
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Chaos Theory as a Model for Managing Issues and Crises.
ERIC Educational Resources Information Center
Murphy, Priscilla
1996-01-01
Uses chaos theory to model public relations situations in which the salient feature is volatility of public perceptions. Discusses the premises of chaos theory and applies them to issues management, the evolution of interest groups, crises, and rumors. Concludes that chaos theory is useful as an analogy to structure image problems and to raise…
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ERIC Educational Resources Information Center
Gutenko, Gregory
Corporate television suffers from at least two "identity crises": departmental isolation, and the lack of a legitimate identity for the corporate video product itself. Video departments are not usually viewed and accepted by the organizational whole as natural evolutions of a historically defined and behaviorally integrated system. The…
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Possible Solutions for Financial Crises of the Private Sector of Higher Education.
ERIC Educational Resources Information Center
Bolling, Landrum R.
Our society is at a point where a number of interlocking crises-inflation, ever rising expectations, war, urban problems, youth's discontent-are coming together. Money is needed at every point and the private college cannot rely on the federal government or private sources to save them from financial disaster. The private college can tackle its…
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2017-01-01
13 Maritime safety ...defense, deterrence of conventional crises and war (through naval readiness and engagement), maritime safety operations, maritime security...measure and to avoid unwanted crises.55 Maritime safety This an area that is primarily the domain of the world’s Coast Guards, but here too the U.S
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Lessons Learned from School Crises and Emergencies, Vol. 1, Issue 2, Fall 2006
ERIC Educational Resources Information Center
US Department of Education, 2006
2006-01-01
"Lessons Learned" is a series of publications that are a brief recounting of actual school emergencies and crises. School and student names have been changed to protect identities. Information for this publication was gathered through a series of interviews with school stakeholders involved in the actual incident. This "Lessons Learned" issue…
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Past Adolescence, into and across Adulthood: Career Crises and Major Decisions
ERIC Educational Resources Information Center
Bakshi, Anuradha J.
2011-01-01
Career-related crises and major decisions, support for these, and job satisfaction were surveyed in 124 varied individuals from Mumbai, India. All participants were in the post-career-entry stage and engaged in paid work; they differed with regard to age (range 18-75 years), sex, marital status, religion, education, occupation, income, and…
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ERIC Educational Resources Information Center
Chesser, Barbara
This bibliography of literature from the 1970s related to crises, family stress, coping, and adaptation contains references of particular interest to professionals in the areas of counseling, education, and family social, psychological and health services. The bibliography is divided into 26 categories; references are classified according to major…