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961.
962.
Mona Jung-König Christoph Füllenbach Michael F. Murphy Paola Manzini Stefan Laspina Kate Pendry Jörg Mühling Agneta Wikman Catherine Humbrecht Jean-Christophe Rigal Sigismond Lasocki Gilles Folléa Erhard Seifried Markus M. Müller Christof Geisen Kari Aranko Kai Zacharowski Patrick Meybohm the PaBloE working group 《Vox sanguinis》2020,115(3):182-191
963.
Séverine Bouttefroy Sandrine Meunier Vanessa Milien Mohamed Boucekine Pierre Chamouni Dominique Desprez Annie Harroche Audrey Hochart Marie Françoise Thiercelin-Legrand Bénédicte Wibaut Hervé Chambost Lucia Rugeri the CoDeC study group 《British journal of haematology》2020,188(2):317-320
This FranceCoag network study assessed 33 patients with congenital factor XIII (FXIII) deficiency presenting FXIII levels <10 iu/dl. Diagnosis was based on abnormal bleeding in 29 patients, a positive family history in 2, recurrent miscarriages in 1 and was fortuitous in 1. Eighteen patients (62·1%) presented life-threatening umbilical or intracranial haemorrhages (ICH). Seven of the 15 patients who experienced ICH were diagnosed but untreated, including 3 with secondary neurological sequelae. All pregnancies without prophylaxis (26/26) led to miscarriages versus 3/16 with prophylaxis. In patients exhibiting FXIII levels <10 iu/dl, prophylaxis could be discussed at diagnosis and at pregnancy. Further controlled prospective studies are needed. 相似文献
964.
Gordon Cook A John Ashcroft Guy Pratt Rakesh Popat Karthik Ramasamy Martin Kaiser Matthew Jenner Sarah Henshaw Rachel Hall Jonathan Sive Simon Stern Matthew Streetly Ceri Bygrave Richard Soutar Neil Rabin Graham H Jackson the United Kingdom Myeloma Forum 《British journal of haematology》2020,190(2):e83-e86
965.
Alexis Régent Brigitte Autran Guislaine Carcelain Rémi Cheynier Benjamin Terrier Bénédicte Charmeteau-De Muylder Alain Krivitzky Eric Oksenhendler Nathalie Costedoat-Chalumeau Pascale Hubert Olivier Lortholary Nicolas Dupin Patrice Debré Lo?c Guillevin Luc Mouthon for the French Idiopathic CD T Lymphocytopenia Study Group 《Medicine》2014,93(2)
Idiopathic CD4 T lymphocytopenia (ICL) is a rare and severe condition with limited available data. We conducted a French multicenter study to analyze the clinical and immunologic characteristics of a cohort of patients with ICL according to the Centers for Disease Control criteria.We recruited 40 patients (24 female) of mean age 44.2 ± 12.2 (19–70) years. Patients underwent T-lymphocyte phenotyping and lymphoproliferation assay at diagnosis, and experiments related to thymic function and interferon (IFN)-γ release by natural killer (NK) cell were performed. Mean follow-up was 6.9 ± 6.7 (0.14–24.3) years. Infectious, autoimmune, and neoplastic events were recorded, as were outcomes of interleukin 2 therapy.In all, 25 patients had opportunistic infections (12 with human papillomavirus infection), 14 had autoimmune symptoms, 5 had malignancies, and 8 had mild or no symptoms. At the time of diagnosis, the mean cell counts were as follows: mean CD4 cell count: 127/mm3 (range, 4–294); mean CD8: 236/mm3 (range, 1–1293); mean CD19: 113/mm3 (range, 3–547); and mean NK cell count: 122/mm3 (range, 5–416). Most patients had deficiency in CD8, CD19, and/or NK cells. Cytotoxic function of NK cells was normal, and patients with infections had a significantly lower NK cell count than those without (p = 0.01). Patients with autoimmune manifestations had increased CD8 T-cell count. Proliferation of thymic precursors, as assessed by T-cell rearrangement excision circles, was increased. Six patients died (15%). CD4 T-cell count <150/mm3 and NK cell count <100/mm3 were predictors of death.In conclusion, ICL is a heterogeneous disorder often associated with deficiencies in CD8, CD19, and/or NK cells. Long-term prognosis may be related to initial CD4 and NK cell deficiency.Abbreviations: AIHA = autoimmune hemolytic anemia, CDC = Centers for Disease Control, CMV = cytomegalovirus, cpm = count per minute, CVID = common variable immunodeficiency, CXCR4 = C-X-C chemokine receptor type 4, HIV = human immunodeficiency virus, HLA = human leukocyte antigen, HPV = human papillomavirus, HTLV-1/2 = human T-cell lymphotropic 1/2, ICL = idiopathic CD4 T lymphocytopenia, IFN-γ = interferon-γ, IL = interleukin, JC virus = John Cunningham virus, LPA = lymphocyte proliferation assay, NK = natural killer, P = patient, PBMC = peripheral blood mononuclear cell, Pwd = pokeweed, SI = stimulation index, sj = signal joint, TREC = T-cell rearrangement excision circle 相似文献
966.
967.
Agnes N Kiragga Judith J Lok Beverly S Musick Ronald J Bosch Ann Mwangi Kara K Wools-Kaloustian Constantin T Yiannoutsos for the East Africa IeDEA Regional Consortium 《Journal of the International AIDS Society》2014,17(1)
Objective
Estimates of CD4 response to antiretroviral therapy (ART) obtained by averaging data from patients in care, overestimate population CD4 response and treatment program effectiveness because they do not consider data from patients who are deceased or not in care. We use mathematical methods to assess and adjust for this bias based on patient characteristics.Design
We examined data from 25,261 HIV-positive patients from the East Africa IeDEA Consortium.Methods
We used inverse probability of censoring weighting (IPCW) to represent patients not in care by patients in care with similar characteristics. We address two questions: What would the median CD4 be “had everyone starting ART remained on observation?” and “were everyone starting ART maintained on treatment?”Results
Routine CD4 count estimates were higher than adjusted estimates even under the best-case scenario of maintaining all patients on treatment. Two years after starting ART, differences between estimates diverged from 30 cells/µL, assuming similar mortality and treatment access among dropouts as patients in care, to over 100 cells/µL assuming 20% lower survival and 50% lower treatment access among dropouts. When considering only patients in care, the proportion of patients with CD4 above 350 cells/µL was 50% adjusted to below 30% when accounting for patients not in care. One-year mortality diverged 6–14% from the naïve estimates depending on assumptions about access to care among lost patients.Conclusions
Ignoring mortality and loss to care results in over-estimation of ART response for patients starting treatment and exaggerates the efficacy of treatment programs administering it. 相似文献968.
Carla Makhlouf Obermeyer Cairn Verhulst Khalil Asmar for the MATCH study group 《Journal of the International AIDS Society》2014,17(1)
Introduction
Although most studies report high frequencies of consent to HIV tests, critics argue that clients are subject to pressure, that acceptors later indicate they could not have refused, and that provider-initiated HIV testing raises serious ethical issues. We examine the meaning of consent and why clients think they could not have refused.Methods
Clients in Burkina Faso, Kenya, Malawi and Uganda were asked about consenting to HIV tests, whether they thought they could have refused and why. Textual responses were analyzed using qualitative and statistical methods.Results
Among 926 respondents, 77% reported they could not have said no, but in fact, 60% actively consented to test, 24% had no objection and only 7% tested without consent. There were few significant associations between categories of consent and their covariates.Conclusions
Retrospectively asking clients if they could have refused to test for HIV overestimates coercion. Triangulating qualitative and quantitative data suggests a considerable degree of agency. 相似文献969.
Ian R Grubb Sarah W Beckham Michel Kazatchkine Ruth M Thomas Eliot R Albers Mauro Cabral Joep Lange Stefano Vella Manoj Kurian Chris Beyrer for the IAS Treatment for Key Affected Populations Working Group 《Journal of the International AIDS Society》2014,17(1)
Introduction
Scientific research has demonstrated the clinical benefits of earlier initiation of antiretroviral treatment (ART), and that ART can markedly reduce HIV transmission to sexual partners. Ensuring universal access to ART for those who need it has long been a core principle of the HIV response, and extending the benefits of ART to key populations is critical to increasing the impact of ART and the overall effectiveness of the HIV response. However, this can only be achieved through coordinated efforts to address political, social, legal and economic barriers that key populations face in accessing HIV services.Discussion
Recent analyses show that HIV prevalence levels among key populations are far higher than among the general population, and they experience a range of biological and behavioural factors, and social, legal and economic barriers that increase their vulnerability to HIV and have resulted in alarmingly low ART coverage. World Health Organization 2014 consolidated guidance on HIV among key populations offers the potential for increased access to ART by key populations, following the same principles as for the general adult population. However, it should not be assumed that key populations will achieve greater access to ART unless stigma, discrimination and punitive laws, policies and practices that limit access to ART and other HIV interventions in many countries are addressed.Conclusions
Rights-based approaches and investments in critical enablers, such as supportive legal and policy environments, are essential to enable wider access to ART and other HIV interventions for key populations. The primary objective of ART should always be to treat the person living with HIV; prevention is an important, additional benefit. ART should be provided only with informed consent. The preventive benefits of treatment must not be used as a pretext for failure to provide other necessary HIV programming for key populations, including comprehensive harm reduction and other prevention interventions tailored to meet the needs of key populations. An end to AIDS is only possible if we overcome the barriers of criminalization, stigma and discrimination that remain key drivers of the HIV epidemics among key populations. 相似文献970.
A multicomponent strategy to improve the availability of antivenom for treating snakebite envenoming
José María Gutiérrez Thierry Burnouf Robert A Harrison Juan J Calvete Ulrich Kuch David A Warrell David J Williams for the Global Snakebite Initiative 《Bulletin of the World Health Organization》2014,92(7):526-532
Snakebite envenoming is a common but neglected public health problem, particularly in impoverished rural regions of sub-Saharan Africa, Asia and Latin America. The only validated treatment for this condition is passive immunotherapy with safe and effective animal-derived antivenoms. However, there is a long-lasting crisis in the availability of these life-saving medications, particularly in sub-Saharan Africa and parts of Asia. We herein advocate a multicomponent strategy to substantially improve the availability of safe and effective antivenoms at the global level. This strategy is based on: (i) preparing validated collections of representative venom pools from the most medically dangerous snakes in high-risk regions of the world; (ii) strengthening the capacity of national antivenom manufacturing and quality control laboratories and their regulatory authorities and establishing new facilities in developing countries through technology transfer, as an integral part of efforts to develop their biological products industry; (iii) getting established laboratories to generate antivenoms for various regions of the world; and (iv) getting governments and relevant organizations to give snakebite envenoming due recognition within national and international public health policy frameworks. These ways of making antivenom available should be complemented by actions to improve health information systems, the accessibility of antivenoms, the training of medical and nursing staff, and community-based education. Such a multicomponent strategy involving stakeholders on many levels could help consolidate sustainable improvements in antivenom availability worldwide. 相似文献