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- Ghaffarpour, N, et al.
(författare)
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Patients with lymphatic malformations who receive the immunostimulant OK-432 experience excellent long-term outcomes
- 2015
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Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 104:11, s. 1169-73
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Tidskriftsartikel (refereegranskat)abstract
- AIM: Sclerotherapy is the primary treatment for lymphatic malformations. The aim of this study was to evaluate the long-term outcome in patients with lymphatic malformations treated with the immunostimulant OK-432 as a sclerosant.METHODS: Between 1998 and 2013, we enrolled 131 of 138 eligible patients treated with OK-432 for lymphatic malformations in a retrospective study. The malformations were categorised according to the International Society for the Study of Vascular Anomalies. The outcome was assessed with a clinical examination and a questionnaire.RESULTS: The lymphatic malformations were localised to the head/neck (60%), the trunk (20%) and the extremities (6%) or involved with more than one region (14%). Patients with microcystic (10%), macrocystic (21%) and mixed lymphatic malformations (69%) underwent a median number of three, two and two injection treatments, respectively. The median age at the first injection was 3.4 years. Good or excellent clinical outcomes were seen in 70% of the patients. The number of injections, previous treatment and lesion localisation, but not time to follow-up and cyst size, predicted the clinical outcome.CONCLUSION: OK-432 treatment resulted in a successful outcome in 70% of patients with lymphatic malformations. The long-term outcome was comparable to the short-term outcome.
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- Nijdam, A., et al.
(författare)
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How to achieve full prophylaxis in young boys with severe haemophilia A: different regimens and their effect on early bleeding and venous access
- 2015
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Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 21:4, s. 444-450
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Tidskriftsartikel (refereegranskat)abstract
- To facilitate early prophylaxis, step-up regimens starting prophylaxis with infusions 1xweek(-1) were introduced. Choice of initial regimen may affect outcome. This study aims to classify initial prophylactic regimens and compare them on short-term outcome. From the European Paediatric Network for Haemophilia Management' (PedNet) registry, patients with severe haemophilia A without inhibitors, born 2000-2012, receiving prophylaxis were included. Treatment centres were classified according to the initial frequency of prophylactic infusions and the age at reaching infusions >= 3 x week(-1). Bleeding, and central venous access device (CVAD) use were compared at age 4 years. In 21 centres with 363 patients, three regimens were identified: (i) start prophylaxis with >= 3 x week(-1) infusions before age three (full: 19% of centres, 18% of patients); (ii) start 1-2 x week(-1), increasing frequency as soon as possible (asap), reaching >= 3 x week(-1) before age three (43% of centres, 36% of patients); (iii) start 1-2 x week(-1), increasing frequency according to bleeding (phenotype), reaching >= 3 x week(-1) after age three (38% of centres, 46% of patients). Prophylaxis was started at median 1.2 years on the full and asap regimen vs 1.8 years on the phenotype regimen. Complete prevention of joint bleeds was most effective on the full regimen (32% full vs. 27% asap and 8% phenotype), though at the cost of using most CVADs (88% full vs. 34% asap and 22% phenotype). The three prophylaxis regimens identified had different effects on early bleeding and CVAD use. This classification provides the first step towards establishing the optimum prophylactic regimen.
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- Steen Carlsson, K, et al.
(författare)
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People with Haemophilia and Female Carriers in Sweden have a Higher Risk of Developing Anxiety, Depression and Pain Based on Treatment Patterns as Compared to Matched Controls: Data from a Registry Study over a Period of 11 Years
- 2019
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Ingår i: ISTH 2019 abstract OC 32.3.
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Konferensbidrag (refereegranskat)abstract
- Background: People with haemophilia (PwH) have increased risk of bleeds associated with acute and chronic pain, and long-term disability. Anxiety and depression are other determinants negatively affecting quality of life in PwH. Despite the severity of these co-morbidities, they have not been extensively investigated and associations between them even less. Aims: The MIND study (NCT03276130) aims to identify patterns of prescribed pain, anti-depressive and anti-anxiety medication and management of pain, depression and anxiety in PwH in four Nordic countries, based on patient registry data (A) and surveys (B). Here we describe current and retrospective treatment patterns of prescribed medication (part A) in PwH in Sweden, explore associations with the PwH background and complication characteristics, and compare prescribed medication use between PwH and the general population. Methods: This 11-year (2007-2017) retrospective population-based registry study, uses international classification systems for diagnoses, surgeries and medications. The study population includes all ages and was identified by diagnosis of haemophilia A or B, or at least one prescription of any factor VIII, IX, or bypassing agent. For each study subject, five age and gender-matched controls were included. Results: Data were extracted for 1550 PwH. The study population is shown in figure 1. The probability of being prescribed with analgesics, neuroleptics and anti-depressants in PwH compared to controls are shown in Table 1. Conclusions : A substantially higher prescription of analgesics in Swedish PwH as compared to controls suggests a need for increased focus on pain management including improved bleed protection and prevention of, e.g. arthropathy. The increased prescription of neurolep-tics and anti- depressants demonstrates that anxiety and depression are overrepresented in PwH and underscores the importance of identifying and managing the broad PwH population including non- frequent health care users. A similar prescription pattern was seen in female carriers suggesting a need for high medical attention and further research to address knowledge gaps.
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