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- Berntorp, Erik, et al.
(författare)
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An approach to study the viral safety of plasma-derived products in previously treated, non-infected patients
- 2001
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Ingår i: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 7:4, s. 360-363
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Tidskriftsartikel (refereegranskat)abstract
- Using the polymerase chain reaction (PCR), we designed a study concept to evaluate the safety of plasma derivatives in previously treated patients who are non-infected by the specific viruses studied. Several product lots can be studied in a single patient, with a study period for each lot of 3 months. In the present study 19 patients were included for treatment with Baxter Hyland Immuno's PCR-screened factor VIII concentrate Immunate (n=7), factor IX concentrate Immunine (n=10), the by-passing agent FEIBA plus Immunine (n=1), and the protein C concentrate Ceprotin (n=1). PCR testing for hepatitis B, C or HIV genomic material in patient samples was done as well as serological testing. All patients remained negative for the tested markers. All seven Immunate patients completed three treatment periods with three different lots of the study drug. The median study period was 282 days and the median dose 115 000 units, with a median of 115 exposure days. Five of the 10 Immunine patients completed three treatment periods and four patients, two treatment periods. One Immunine patient was discontinued from the study for reasons unrelated to the study drug administration. The median study period was 305 days and the median total dose 82 200 units, with a median of 88 exposure days. Our study presents a new design to approach the evaluation of viral safety of new plasma derivatives in previously treated, non-infected patients (NIPs) and offers several advantages over the currently recommended studies using testing for serological markers of infection in previously untreated patients (PUPs).
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| 3. |
- Graziani, F., et al.
(författare)
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Oral Care and Quality Evaluation: A Multicentric Study on Periodontal Treatment
- 2020
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Ingår i: Oral Health & Preventive Dentistry. - 1602-1622. ; 18:2, s. 363-371
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: No information is available on the perception of the quality of care in patients treated for periodontitis. The purpose of this article was to assess how periodontitis-affected patients perceive the quality of periodontal treatment (PT) and to measure the factors which may influence it. Materials and Methods: 306 subjects who completed PT were invited to participate. Questionnaires and visual analogic scales (VAS) evaluating perception of quality of care, symptoms, and oral health related quality of life (OHRQoL) were handed out. Oral and periodontal indicators were collected before and after treatment. The impact of different factors on perception of quality was assessed with a regression model. Results: Quality evaluation was high yet unrelated for both patients and clinicians (p = 0.983). Quality was negatively influenced by the number of residual oral infections (p < 0.001), patient's age (p = 0.07) and presence of residual pain at completion of PT (p = 0.02). Professionalism, kindness of the staff and communication skills were the characteristics mostly appreciated. The OHRQoL was influenced by the number of residual teeth (p < 0.001), increasing age of patients (p = 0.08), number of residual infections (p < 0.01) and pain (p = 0.04). Conclusions: Patients' quality perception appeared to be influenced by clinical and emotional aspects. Oral care providers should be aware of the impact of non-clinical factors in patients' appreciation of quality of treatment.
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| 4. |
- Berntorp, Erik, et al.
(författare)
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von Willebrand's disease: a report from a meeting in the Åland islands.
- 2012
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Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 18 Suppl 6, s. 1-13
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Tidskriftsartikel (refereegranskat)abstract
- von Willebrand's disease (VWD) is probably the most common bleeding disorder, with some studies indicating that up to 1% of the population may have the condition. Over recent years interest in VWD has fallen compared to that of haemophilia, partly the result of focus on blood-borne diseases such as HIV and hepatitis. Now the time has come to revisit VWD, and in view of this some 60 international physicians with clinical and scientific interest in VWD met over 4 days in 2010 in the Åland islands to discuss state-of-the-art issues in the disease. The Åland islands are where Erik von Willebrand had first observed a bleeding disorder in a number of members of a family from Föglö, and 2010 was also the 140th anniversary of his birth. This report summarizes the main papers presented at the symposium; topics ranged from genetics and biochemistry through to classification of VWD, pharmacokinetics and laboratory assays used in the diagnosis of the disease, inhibitors, treatment guidelines in different age groups including the elderly who often have comorbid conditions that present challenges, and prophylaxis. Other topics included managing surgeries in patients with VWD and the role of FVIII in VWF replacement, a controversial subject.
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| 13. |
- Knobe, Karin, et al.
(författare)
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Factor VIII inhibitors in two families with mild haemophilia A: structural analysis of the mutations
- 2000
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Ingår i: Haemostasis. - : S. Karger AG. - 0301-0147. ; 30:5, s. 268-279
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Tidskriftsartikel (refereegranskat)abstract
- The development of inhibitory antibodies against coagulation factor VIII (FVIII) in patients with mild haemophilia A is uncommon. We describe here two families in which three or two members have developed inhibitors, suggesting a familial predisposition. The mutations found, in the A2 (Arg593Cys) and C1 domains (Tyr2105Cys), have been reported to give rise to inhibitor development in single individuals in addition to the family cluster we describe, strongly suggesting that these amino acid substitutions give rise to a more immunogenic protein. The analysis of structural models of activated factor VIII revealed that Arg593 is solvent-exposed and involved in a network of electrostatic interactions while Tyr2105 is partially buried and has hydrophobic interactions essentially with Ile2144. All these residues are strictly conserved in the FVIII amino acid sequence from man, pig and mouse, suggesting, at least, that they have structural roles. We propose that the two mutations in these families could cause mild haemophilia A because they induce local conformational changes (and possible secretion or intermolecular interaction problems, e.g., with von Willebrand factor) compatible with immunogenicity and production of inhibitors against the infused wild-type FVIII.
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| 14. |
- Ljung, R., et al.
(författare)
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Treatment of children with haemophilia in Europe: A survey of 20 centres in 16 countries
- 2000
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Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 6, s. 619-624
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Tidskriftsartikel (refereegranskat)abstract
- A survey was made of the current status of treatment of haemophilic boys at 20 centres in 16 European countries and includes approximately 1500 of the estimated 6500 haemophiliacs in the participating countries. Many mild haemophiliacs are not seen, or seen infrequently, at haemophilia centres and this requires study. Nine of 18 centres provide continuous prophylaxis to 80-100% of their patients, five centres provide it to 55-80% and the remaining four centres to 15-40% of the boys. The median dose given was 6240 U kg-1 year-1 (range 3120-7800). Four centres administered only recombinant concentrates to children with severe haemophilia A, while seven centres administered recombinant concentrates to 75-90% and the remaining centres to less than 50% of the boys (two centres <10%). When asked for the choice of concentrate for a newly diagnosed boy with severe haemophilia A, all but one centre preferred recombinant concentrate. Most boys below 6 years received concentrates via a peripheral vein but three centres preferred a central venous line for 80-100% of the boys. Thirteen of 18 centres applied home treatment to 84-100% of the boys and the remaining five centres to 57-77% of the boys.
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| 15. |
- Loth, Daan W, et al.
(författare)
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Genome-wide association analysis identifies six new loci associated with forced vital capacity
- 2014
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Ingår i: Nature Genetics. - : Springer Science and Business Media LLC. - 1061-4036 .- 1546-1718. ; 46, s. 669-677
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Tidskriftsartikel (refereegranskat)abstract
- Forced vital capacity (FVC), a spirometric measure of pulmonary function, reflects lung volume and is used to diagnose and monitor lung diseases. We performed genome-wide association study meta-analysis of FVC in 52,253 individuals from 26 studies and followed up the top associations in 32,917 additional individuals of European ancestry. We found six new regions associated at genome-wide significance (P < 5 × 10(-8)) with FVC in or near EFEMP1, BMP6, MIR129-2-HSD17B12, PRDM11, WWOX and KCNJ2. Two loci previously associated with spirometric measures (GSTCD and PTCH1) were related to FVC. Newly implicated regions were followed up in samples from African-American, Korean, Chinese and Hispanic individuals. We detected transcripts for all six newly implicated genes in human lung tissue. The new loci may inform mechanisms involved in lung development and the pathogenesis of restrictive lung disease.
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