| 1. |
- 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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| 2. |
- Alfaro-Murillo, A, et al.
(författare)
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Selective IgA deficiency, juvenile idiopathic arthritis and anterior uveitis in a Costa Rican child. Coincidental diseases?. Case report and literature review
- 2022
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Ingår i: Revista Ciencias Biomédicas. ; 11:3, s. 243-249.
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Tidskriftsartikel (refereegranskat)abstract
- Background: selective IgA deficiency is the most frequent primary immunodeficiency worldwide. Patients are usually asymptomatic. However, those cases with symptoms develop recurrent infections and increased risk of autoimmune and malignant diseases. On the other hand, rheumatic disorders are uncommon during childhood with juvenile idiopathic arthritis as the most common one. Case Presentation: we present the case of a female patient, who developed oligoarticular juvenile idiopathic arthritis at age 7 years. After the diagnosis, she developed acute anterior uveitis. During the initial immunological evaluation, the diagnosis of selective IgA deficiency was confirmed. A work-up for immunodeficiency demonstrated a normal T cell compartment. B cell subpopulations showed normal memory B lymphocytes, absence of transitional B cells, and an increase in the CD21 low unique subset. Conclusions: at the beginning of any rheumatological evaluation, the physician should request immunoglobulins levels, in order to detect possible primary antibodies deficiencies.
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| 3. |
- Arnstad, Ellen Dalen, et al.
(författare)
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Fatigue in young adults with juvenile idiopathic arthritis 18 years after disease onset : data from the prospective Nordic JIA cohort
- 2021
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Ingår i: Pediatric Rheumatology. - : BioMed Central (BMC). - 1546-0096. ; 19:1
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Tidskriftsartikel (refereegranskat)abstract
- Background To study fatigue in young adults with juvenile idiopathic arthritis (JIA) 18 years after disease onset, and to compare with controls. Methods Consecutive children with onset of JIA between 1997 and 2000, from geographically defined areas of Norway, Sweden, Denmark and Finland were followed for 18 years in a close to population-based prospective cohort study. Clinical features, demographic and patient-reported data were collected. Inclusion criteria in the present study were a baseline visit 6 months after disease onset, followed by an 18-year follow-up with available self-reported fatigue score (Fatigue Severity Scale (FSS), 1-7). Severe fatigue was defined as FSS >= 4. For comparison, Norwegian age and sex matched controls were used. Results Among 377 young adults with JIA, 26% reported severe fatigue, compared to 12% among controls. We found higher burden of fatigue among participants with sleep problems, pain, poor health, reduced participation in school/work, physical disability, active disease, or use of disease-modifying anti-rheumatic drugs (DMARDs)/biologics/systemic steroids. In contrast, participants without these challenges, had fatigue scores similar to controls. Active disease assessed at all three time points (baseline, 8-year and 18-year follow-up) was associated with higher mean fatigue score and higher percentage of severe fatigue compared to disease courses characterized by periods of inactive disease. Predictors of fatigue at the 18-year follow-up were female sex and diagnostic delay of >= 6 months at baseline, and also pain, self-reported poor health, active disease, and previous/ongoing use of DMARDs/biologics at 8 years. Conclusions Fatigue is a prominent symptom in young adults with JIA, with higher fatigue burden among participants with poor sleep, pain, self-reported health problems, active disease, or use of DMARDs/biologics. Participants without these challenges have results similar to controls. Patient- and physician-reported variables at baseline and during disease course predicted fatigue at 18-year follow-up.
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| 4. |
- Brix, Ninna, et al.
(författare)
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M-ficolin: a valuable biomarker to identify leukaemia from juvenile idiopathic arthritis.
- 2022
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Ingår i: Archives of disease in childhood. - : BMJ. - 1468-2044 .- 0003-9888. ; 107:4, s. 371-376
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Tidskriftsartikel (refereegranskat)abstract
- Distinction on clinical grounds between acute lymphoblastic leukaemia presenting with arthropathy (ALLarthropathy) and juvenile idiopathic arthritis (JIA) is difficult, as the clinical and paraclinical signs of leukaemia may be vague. The primary aim was to examine the use of lectin complement pathway proteins as markers to differentiate ALLarthropathy from JIA. The secondary aims were to compare the protein levels at baseline and follow-up in a paired number of children with ALL and to examine the correlation with haematology counts, erythrocyte sedimentation reaction (ESR), C-reactive protein (CRP), blasts, relapse and death.In this observational study, we measured M-ficolin, CL-K1 and MASP-3 in serum from children with ALL (n=151) and JIA (n=238) by time-resolved immunofluorometric assays. Logistic regression was used for predictions of ALL risk, considering the markers as the respective exposures. We performed internal validation using repeated '10-fold cross-validation' with 100 repetitions computing the area under the curve (AUC) as well as positive and negative predictive values in order to evaluate the predictive performance.The level of M-ficolin was higher in JIA than ALLtotal and the ALLarthropathy subgroup. The M-ficolin level normalised after remission of ALL. M-ficolin could differentiate ALL from JIA with an AUC of 94% and positive predictive value (PPV) of 95%, exceeding CRP and haemoglobin. In a dichotomised predictive model with optimal cut-offs for M-ficolin, platelets and haemoglobin, AUC was 99% and PPV 98% in detecting ALL from JIA.M-ficolin is a valuable marker to differentiate the child with ALL from JIA.
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| 5. |
- Fasth, Anders, 1945
(författare)
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Covid-19 hos barn med immunbrist – kan det lära oss något om viruset och immunförsvaret?
- 2021
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Ingår i: Barnläkaren. - 1651-0534. ; :3, s. 14-15
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Inledningsvis i pandemin var inställningen att barn med primära immunbrister (PID) löper en hög risk för att få allvarlig covid-19 infektion. Detta oavsett om barnet har T-cellsdefekt, B-cellsdefekt eller en defekt i det medfödda immunförsvaret. Men är det verkligen så?
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| 6. |
- Fasth, Anders, 1945, et al.
(författare)
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Lars-Åke Hanson: In Memoriam.
- 2022
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Ingår i: Annals of nutrition & metabolism. - : S. Karger AG. - 1421-9697 .- 0250-6807. ; 78:6, s. 361-362
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)
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| 7. |
- Felber, M., et al.
(författare)
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Targeted busulfan-based reduced-intensity conditioning and HLA-matched HSCT cure hemophagocytic lymphohistiocytosis
- 2020
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Ingår i: Blood Advances. - : American Society of Hematology. - 2473-9529 .- 2473-9537. ; 4:9, s. 1998-2010
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Tidskriftsartikel (refereegranskat)abstract
- Reduced-intensity/reduced-toxicity conditioning and allogeneic T-cell replete hematopoietic stem cell transplantation are curative in patients with hemophagocytic lymphohistiocytosis (HLH). Unstable donor chimerism (DC) and relapses are clinical challenges. We examined the effect of a reduced-intensity conditioning regimen based on targeted busulfan to enhance myeloid DC in HLH. The European Society for Bone and Marrow Transplantation-approved reduced-intensity conditioning protocol comprised targeted submyeloablative IV busulfan, IV fludarabine, and serotherapy comprising IV alemtuzumab (0.5-0.8 mg/kg) for unrelated-donor and IV rabbit anti-T-cell globulin for related-donor transplants. We assessed toxicity, engraftment, graft-versus-host disease (GHVD), DC in blood cell subtypes, and overall survival/event-free survival. Twenty-five patients from 7 centers were treated (median age, 0.68 year). The median total dose and cumulative area under the curve of busulfan was 13.1 mg/kg (6.4-26.4) and 63.1 mg/L x h (48-77), respectively. Bone marrow, peripheral blood stem cell, or cord blood transplants from HLA-matched related (n = 7) or unrelated (n = 18) donors were administered. Donor cells engrafted in all patients (median: neutrophils d+20/platelets d128). At last follow-up (median, 36 months; range, 8-111 months), the median DC of CD151 neutrophils, CD3(+) T cells, and CD16(+)56(+) natural killer cells was 99.5% (10-100), 97% (30-100), and 97.5% (30-100), respectively. Eight patients (32%) developed sinusoidal obstruction syndrome, resolving after defibrotide treatment. The 3-year overall survival and event-free survival rates were both 100%. None of the patients developed acute grade III to IV GHVD. Limited chronic GVHD was encountered in 4%. This regimen achieves excellent results with stable DC in patients with HLH.
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| 8. |
- Fernandes, J. F., et al.
(författare)
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Outcomes after Haploidentical Stem Cell Transplantation with Post-Transplantation Cyclophosphamide in Patients with Primary Immunodeficiency Diseases
- 2020
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Ingår i: Biology of Blood and Marrow Transplantation. - : Elsevier BV. - 1083-8791. ; 26:10, s. 1923-1929
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Tidskriftsartikel (refereegranskat)abstract
- Allogeneic hematopoietic stem cell transplantation (HCT) can cure primary immunodeficiency diseases (PID). When a HLA-matched donor is not available, a haploidentical family donor may be considered. The use of T cell-replete haploidentical HCT with post-transplantation cyclophosphamide (haplo-PTCy) in children with PID has been reported in few case series. A donor is usually readily available, and haplo-PTCy can be used in urgent cases. We studied the outcomes of 73 patients with PID who underwent haplo-PTCy, including 55 patients who did so as a first transplantation and 18 who did so as a salvage transplantation after graft failure of previous HCT. The median patient age was 1.6 years. Most of the children were male (n = 54) and had active infection at the time of transplantation (n = 50); 10 children had severe organ damage. The diagnosis was severe combined immunodeficiency (SCID) in 34 patients and non-SCID in 39 (Wiskott-Aldrich syndrome; n = 14; chronic granulomatous disease, n = 10; other PID, n = 15). The median duration of follow-up of survivors was 2 years. The cumulative incidence of neutrophil recovery was 88% in the SCID group and 84% in non-SCID group and was 81% for first transplantations and 83% after a salvage graft. At 100 days, the cumulative incidence of acute GVHD grade II-IV and III-IV was 33% and 14%, respectively. The majority of patients reached 200/mu L CD4(+) and 1000/mu L CD3(+) cell counts between 3 and 6 months. The estimated 2-year overall survival was 66%; it was 64% for SCID patients and 65% for non-SCID patients and 63% for first HCT and 77% for salvage transplantations. Twenty-five patients died, most of them due to infection early after transplantation (before 100 days). In conclusion, haplo-PTCy is a feasible procedure, can cure two-thirds of children with PID, and can be used as rescue treatment for previous graft failure. (C) 2020 American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc. (c) 2020 American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc.
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| 9. |
- Fernell, Elisabeth, 1948, et al.
(författare)
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Paediatric Acute onset Neuropsychiatric Syndrome: Exploratory study finds no evidence of HLA class II association but high rate of autoimmunity in first-degree relatives
- 2022
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Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 11:4, s. 820-824
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Tidskriftsartikel (refereegranskat)abstract
- Aim Paediatric acute-onset neuropsychiatric syndrome (PANS) is defined by an acute onset of obsessive-compulsive disorder and/or eating restrictions and at least two other severe neuropsychiatric symptoms. The condition is suspected to have an immune-mediated pathophysiology, but reliable biomarkers have not been identified. Methods We hypothesised that PANS, like narcolepsy, might have a human leucocyte antigen (HLA) association, as found in 95% of children developing narcolepsy after H1N1 immunisation. Low resolution genotyping of the MHC class II antigens HLA-DRB1 and HLA-DQB1 was performed using two different PCR-based methods. In addition, parents were interviewed regarding a detailed family history of autoimmune diseases in first-degree relatives. A total of 18 children, aged 5-14 (mean 8.2) years at onset of PANS met symptom criteria. Results No evident association between PANS and the specific HLA alleles examined was observed. In first-degree relatives of 10 of the 18 children, an autoimmune disease had been diagnosed, and three of the 18 children themselves had an autoimmune disease. Conclusion No HLA allele association such as seen in children with narcolepsy after H1N1 immunisation could be confirmed in this group of children with PANS. However, more than half the group had a first-degree relative with a diagnosed autoimmune disease.
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| 10. |
- Glerup, Mia, et al.
(författare)
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Changing patterns in treatment, remission status and categories in a long-term Nordic cohort study of juvenile idiopathic arthritis.
- 2022
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Ingår i: Arthritis care & research. - : Wiley. - 2151-4658 .- 2151-464X.
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Tidskriftsartikel (refereegranskat)abstract
- To explore sustainability of achieved remission off medication and defined ILAR categories in juvenile idiopathic arthritis (JIA). To describe the trajectory of disease course over time by comparing treatment, disease activity and ILAR categories from baseline, 8 and 18 years of disease.Included were 373 of the 510 initially recruited consecutive cases of JIA from the prospective longitudinal, population-based Nordic JIA cohort with disease onset during 1997-2000 from Denmark, Norway, Sweden, and Finland in an 18-year follow-up study. Clinical data was collected consecutively at baseline, 8 and 18 years of disease, and evaluated regarding treatment, disease activity and ILAR category.Significantly more patients (70%) were off medication after 18 years of follow-up compared to after 8 years (59.7%); nevertheless, the number of patients in remission had not increased (52% versus 51%). Twelve percent of patients changed ILAR category between 8 and 18 years after disease onset. Almost half of the changes were due to updated information about heredity in a first degree relative. In the same period, the psoriatic group increased significantly in number (p<0.001) contrasting the oligoarticular category, which decreased (p=0.02). The undifferentiated group increased 24% from 8 to 18 years, however, this was not significant (p=0.06).In this Nordic JIA cohort study the remission rate did not increase even though significantly more patients were off medication at the 18-year follow-up compared to 8 years after disease onset. The distribution of patients in the ILAR categories continued to change significantly throughout the 18-year study period.
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