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| 2. |
- Ahlin, Anders, et al.
(författare)
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Chronic granulomatous disease - haematopoietic stem cell transplantation versus conventional treatment.
- 2013
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Ingår i: Acta paediatrica (Oslo, Norway : 1992). - : Wiley. - 1651-2227 .- 0803-5253. ; 102:11, s. 1087-1094
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Tidskriftsartikel (refereegranskat)abstract
- Chronic granulomatous disease (CGD) is a rare X-linked or autosomal recessive primary immune deficiency characterized by recurrent, life-threatening bacterial and fungal infections. Mortality rates are high with conventional treatment. However, haematopoietic stem cell transplantation (HSCT) offers cure. Here, we compare the outcome of HSCT in 14 Swedish patients with CGD to that in 27 patients with CGD who were given conventional treatment.
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| 4. |
- Gadalla, Shahinaz M, et al.
(författare)
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Outcomes of allogeneic hematopoietic cell transplant in patients with dyskeratosis congenita.
- 2013
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Ingår i: Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation. - : Elsevier BV. - 1523-6536. ; 19:8, s. 1238-1243
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Tidskriftsartikel (refereegranskat)abstract
- We describe outcomes after allogeneic transplantation in 34 patients with dyskeratosis congenita transplanted between 1981 and 2009. The median age at transplantation was 13 years (range 2 - 35). Approximately 50% of transplants were from related donors. Bone marrow was the predominant source of stem cells (n=24/34). The day-28 probability of neutrophil recovery was 73% and the day-100 platelet recovery was 72%. The day-100 probability of grade II-IV acute GVHD and the 3-year probability of chronic GVHD were 24% and 37%, respectively. The 10-year probability of survival was 30%; 14 patients were alive at last follow-up. Ten deaths occurred within 4 months from transplantation due to graft failure (n=6) or other transplant-related complications; 9 of these patients had been transplanted from mismatched related or from unrelated donors. Another 10 deaths occurred after 4 months; 6 of them occurred more than 5 years from transplantation, 4 of these were attributed to pulmonary failure. Transplant-regimen intensity and transplants from mismatched related or unrelated donors were associated with early mortality. Transplantation of grafts from HLA-matched siblings with cyclophosphamide-containing non-radiation regimens was associated with early low toxicity. Late mortality was attributed mainly to pulmonary complications and likely related to the underlying disease.
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| 5. |
- Glerup, Mia, et al.
(författare)
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Inflammatory biomarkers predicting long-term remission and active disease in juvenile idiopathic arthritis: a population-based study of the Nordic JIA cohort
- 2024
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Ingår i: RMD OPEN. - 2056-5933. ; 10:3
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Tidskriftsartikel (refereegranskat)abstract
- Objectives To assess the ability of baseline serum biomarkers to predict disease activity and remission status in juvenile idiopathic arthritis (JIA) at 18-year follow-up (FU) in a population-based setting.Methods Clinical data and serum levels of inflammatory biomarkers were assessed in the longitudinal population-based Nordic JIA cohort study at baseline and at 18-year FU. A panel of 16 inflammatory biomarkers was determined by multiplexed bead array assay. We estimated both univariate and multivariate logistic regression models on binary outcomes of disease activity and remission with baseline variables as explanatory variables.Results Out of 349 patients eligible for the Nordic JIA cohort study, 236 (68%) had available serum samples at baseline. We measured significantly higher serum levels of interleukin 1 beta (IL-1 beta), IL-6, IL-12p70, IL-13, MMP-3, S100A9 and S100A12 at baseline in patients with active disease at 18-year FU than in patients with inactive disease. Computing receiver operating characteristics illustrating the area under the curve (AUC), we compared a conventional prediction model (gender, age, joint counts, erythrocyte sedimentation rate, C reactive protein) with an extended model that also incorporated the 16 baseline biomarkers. Biomarker addition significantly improved the ability of the model to predict activity/inactivity at the 18-year FU, as evidenced by an increase in the AUC from 0.59 to 0.80 (p=0.02). Multiple regression analysis revealed that S100A9 was the strongest predictor of inactive disease 18 years after disease onset.Conclusion Biomarkers indicating inflammation at baseline have the potential to improve evaluation of disease activity and prediction of long-term outcomes.
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| 7. |
- Milerad, Josef, 1947, et al.
(författare)
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Vaccinera barn från fem år snarast
- 2021
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Ingår i: Svenska Dagbladet. - 1101-2412. ; :2021-12-16
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Tidskriftsartikel (populärvet., debatt m.m.)
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| 8. |
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| 9. |
- Wekell, Per, et al.
(författare)
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An overview of how on-call consultant paediatricians can recognise and manage severe primary immunodeficiencies.
- 2019
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Ingår i: Acta paediatrica. - : Wiley. - 1651-2227 .- 0803-5253. ; 108:12, s. 2175-2185
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Tidskriftsartikel (refereegranskat)abstract
- Severe primary paediatric immunodeficiency syndromes are rare and potentially fatal unless suspected, diagnosed and treated early. We provide clinical guidance and support for on-call consultant paediatricians working in secondary level hospitals on how to recognise and manage children with these conditions. Our paper addresses four conditions that risk the most severe outcomes if they are not adequately cared for during on-call periods, such as weekends: severe combined immunodeficiency, haemophagocytic lymphohistiocytosis, severe congenital neutropaenia and chronic granulomatous disease. CONCLUSION: On-call paediatricians are provided with advice on handling the most severe primary immunodeficiencies.
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| 10. |
- Wekell, Per, et al.
(författare)
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Fifteen-minute consultation: Recognising primary immune deficiencies in children
- 2019
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Ingår i: Archives of Disease in Childhood: Education and Practice Edition. - : BMJ. - 1743-0585 .- 1743-0593. ; 104, s. 235-243
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Tidskriftsartikel (refereegranskat)abstract
- © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ. Children with primary immunodeficiency syndromes present with broad variation of clinical features and the consequences are often severe if not promptly recognised. Here, support is provided for the general paediatrician to recognise primary immunodeficiencies among the many children they meet in their clinical practice.
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