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- Vlachos, Adrianna, et al.
(författare)
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Diagnosing and treating Diamond Blackfan anaemia : results of an international clinical consensus conference
- 2008
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Ingår i: British Journal of Haematology. - : Wiley. - 0007-1048 .- 1365-2141. ; 142:6, s. 859-876
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Forskningsöversikt (refereegranskat)abstract
- Diamond Blackfan anaemia (DBA) is a rare, genetically and clinically heterogeneous, inherited red cell aplasia. Classical DBA affects about seven per million live births and presents during the first year of life. However, as mutated genes have been discovered in DBA, non-classical cases with less distinct phenotypes are being described in adults as well as children. In caring for these patients it is often difficult to have a clear understanding of the treatment options and their outcomes because of the lack of complete information on the natural history of the disease. The purpose of this document is to review the criteria for diagnosis, evaluate the available treatment options, including corticosteroid and transfusion therapies and stem cell transplantation, and propose a plan for optimizing patient care. Congenital anomalies, mode of inheritance, cancer predisposition, and pregnancy in DBA are also reviewed. Evidence-based conclusions will be made when possible; however, as in many rare diseases, the data are often anecdotal and the recommendations are based upon the best judgment of experienced clinicians. The recommendations regarding the diagnosis and management described in this report are the result of deliberations and discussions at an international consensus conference.
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| 2. |
- Abuzeid, Nadir, et al.
(författare)
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Antimycobacterial activity of selected medicinal plants traditionally used in Sudan to treat infectious diseases
- 2014
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Ingår i: Journal of Ethnopharmacology. - : Elsevier. - 0378-8741 .- 1872-7573. ; 157, s. 134-139
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Tidskriftsartikel (refereegranskat)abstract
- Ethnopharmacological relevance: The emergence of multidrug-resistant strains of Mycobacterium tuberculosis underscores the need for continuous development of new and efficient methods to determine the susceptibility of isolates of Mycobacterium tuberculosis in the search for novel antimycobacterial agents. Natural products constitute an important source of new drugs, and design and implementation of antimycobacterial susceptibility testing methods are necessary to evaluate the different extracts and compounds. In this study we have explored the antimycobacterial properties of 50 ethanolic extracts from different parts of 46 selected medicinal plants traditionally used in Sudan to treat infectious diseases. Materials and methods: Plants were harvested and ethanolic extracts were prepared. For selected extracts, fractionation with hydrophilic and hydrophobic solvents was undertaken. A luminometry-based assay was used for determination of mycobacterial growth in broth cultures and inside primary human macrophages in the presence or absence of plant extracts and fractions of extracts. Cytotoxicity was also assessed for active fractions of plant extracts. Results: Of the tested extracts, three exhibited a significant inhibitory effect on an avirulent strain of Mycobacterium tubercluosis (H37Ra) at the initial screening doses (125 and 6.25 mu g/ml). These were bark and leaf extracts of Khaya senegalensis and the leaf extract of Rosmarinus officinalis L. Further fractions of these plant extracts were prepared with n-hexane, chloroform, ethyl acetate, n-butanol, ethanol and water, and the activity of these extracts was retained in hydrophobic fractions. Cytotoxicity assays revealed that the chloroform fraction of Khaya senegalensis bark was non-toxic to human monocyte-derived macrophages and other cell types at the concentrations used and hence, further analysis, including assessment of IC50 and intracellular activity was done with this fraction. Conclusion: These results encourage further investigations to identify the active compound(s) within the chloroform fraction of Khaya senegalensis bark. (C) 2014 Elsevier Ireland Ltd. All rights reserved.
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| 6. |
- Glader, A, et al.
(författare)
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Byggnadsrelaterad ohälsa : Kompetensutveckling inom hälsovården
- 2014
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Rapport (refereegranskat)abstract
- Det finns ett stort behov av utbildning om inomhusmiljö och byggnadsrelaterad ohälsa, både inom ramen för grundläggande yrkesutbildningar och som fortbildningskurser för yrkesverksamma. Hälsoproblem som beror på dålig inomhusluft på arbetsplatsen utreds av företagshälsovården. Dock har de som jobbar inom företagshälsovård idag inte alltid tillräcklig kunskap om inomhusmiljö och byggnadsrelaterad ohälsa och saknar ofta beredskap att som sakkunniga delta i utredningar på arbetsplatser. För att förbättra vården bör personalen utbildas om bl.a. riskfaktorer för dålig inomhusluft, vanliga hälsobesvär och inverkan på arbetsförmåga och produktivitet, de psykosociala faktorernas betydelse samt ansvarsfrågor och hälsoekonomiska konsekvenser. Öppna digitala lärresurser (OER) och kurser på nätet (MOOCs) kan med fördel användas vid fortbildning av vårdpersonal. Nätbaserad utbildning underlättar för vårdpersonalen att själv kunna välja tidpunkt och målsättning. Därtill utgör OER även ett hjälpmedel för att utveckla kunskapsöverföringen mellan Sverige och Finland.
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| 9. |
- Ostadkarampour, M., et al.
(författare)
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Higher levels of interleukin IL-17 and antigen-specific IL-17 responses in pulmonary sarcoidosis patients with Lofgren's syndrome
- 2014
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Ingår i: Clinical and Experimental Immunology. - : Oxford University Press (OUP). - 0009-9104 .- 1365-2249. ; 178:2, s. 342-352
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Tidskriftsartikel (refereegranskat)abstract
- Sarcoidosis is a granulomatous disorder of unknown aetiology. The presence of Mycobacterium tuberculosis catalase-peroxidase (mKatG) in sarcoidosis tissue has been reported. T helper type 1 (Th1) responses against mKatG have previously been observed. However, little is known about interleukin (IL)-17 and Th17 responses in sarcoidosis. Here, we investigated the levels of IL-17 and frequencies of IL-17-producing cells responding to mKatG in sarcoidosis patients with different prognosis. Peripheral blood and bronchoalveolar lavage (BAL) cells were obtained from sarcoidosis patients with or without Lofgren's syndrome (often associated with spontaneous recovery), and also stratified according to human leucocyte antigen (HLA) type. Cells producing IL-17 and interferon (IFN)-gamma after stimulation with mKatG were enumerated by enzyme-linked immunospot (ELISPOT). The level of IL-17 in the BAL fluid of sarcoidosis patients and healthy controls was measured by quantitative immuno-polymerase chain reaction (qIPCR). We also performed flow cytometry and immunohistochemistry for further characterization of IL-17 expression. Patients with Lofgren's syndrome had a higher frequency of IL-17-producing cells responding to mKatG in BAL fluid compared to patients without Lofgren's syndrome (P < 0.05). The HLA-DR3(+) sarcoidosis patients with Lfgren's syndrome (known to have a particularly good prognosis) also had a clearly higher level of IL-17 in BAL fluid compared to healthy controls and sarcoidosis patients without Lofgren's syndrome (P < 0.01) and (P < 0.05), respectively. No such difference between patient groups was observed with regard to IFN-gamma and not with regard to either cytokine in peripheral blood. These findings suggest that IL-17-producing cells may be a useful biomarker for the prognosis of sarcoidosis and play a role in the spontaneous recovery typical of patients with Lfgren's syndrome.
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| 10. |
- Wilkes, Mark C., et al.
(författare)
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Metformin-induced suppression of Nemo-like kinase improves erythropoiesis in preclinical models of Diamond–Blackfan anemia through induction of miR-26a
- 2020
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Ingår i: Experimental Hematology. - : Elsevier BV. - 0301-472X. ; 91, s. 65-77
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Tidskriftsartikel (refereegranskat)abstract
- Diamond–Blackfan anemia (DBA) results from haploinsufficiency of ribosomal protein subunits in hematopoietic progenitors in the earliest stages of committed erythropoiesis. Nemo-like kinase (NLK) is chronically hyperactivated in committed erythroid progenitors and precursors in multiple human and murine models of DBA. Inhibition of NLK activity and suppression of NLK expression both improve erythroid expansion in these models. Metformin is a well-tolerated drug for type 2 diabetes with multiple cellular targets. Here we demonstrate that metformin improves erythropoiesis in human and zebrafish models of DBA. Our data indicate that the effects of metformin on erythroid proliferation and differentiation are mediated by suppression of NLK expression through induction of miR-26a, which recognizes a binding site within the NLK 3′ untranslated region (3′UTR) to facilitate transcript degradation. We propose that induction of miR-26a is a potentially novel approach to treatment of DBA and could improve anemia in DBA patients without the potentially adverse side effects of metformin in a DBA patient population.
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