| 51. |
- Lundkvist, Annika, et al.
(författare)
-
Functional performance in self-care and mobility after selective dorsal rhizotomy : a 10-year practice-based follow-up study.
- 2015
-
Ingår i: Developmental Medicine & Child Neurology. - : Wiley. - 0012-1622 .- 1469-8749. ; 57:3, s. 286-293
-
Tidskriftsartikel (refereegranskat)abstract
- AIM: To explore changes in performance in daily activities (self-care and mobility) 10 years after selective dorsal rhizotomy (SDR).METHOD: Twenty-four children with bilateral spastic cerebral palsy were followed; the median age at SDR was 4 years 1 month (range 2y 5mo-6y 4mo) and at 10-year follow-up was 14 years 6 months (range 12y 3mo-16y 9mo). The preoperative Gross Motor Function Classification System (GMFCS) levels were: I (n=1), II (n=7), III (n=4), IV (n=11), and V (n=1). The Pediatric Evaluation of Disability Inventory (PEDI) was used to assess performance in functional skills, caregiver assistance, and frequency of modifications and adaptive equipment (MAE) in self-care and mobility domains. Changes were analysed in relation to preoperative GMFCS levels, PEDI scores, and age at operation.RESULTS: All scores improved significantly (p<0.01) during the first 5 years in patients assigned to GMFCS levels I-III and IV-V. Between 5 years and 10 years, changes were seen in patients grouped in GMFCS levels I-III in the functional skills, mobility (p=0.04), caregiver assistance self-care (p=0.03), and caregiver assistance mobility (p=0.03) domains. Those grouped in GMFCS levels IV-V showed small changes between 5 years and 10 years after surgery. Changes were dependent on the preoperative GMFCS levels in all domains; caregiver assistance, self-care and mobility changes were dependent on preoperative values. The use of MAE increased in participants in GMFCS levels IV-V.INTERPRETATION: Children who underwent SDR and physiotherapy improved in functional performance in self-care and mobility and were more independent 10 years postoperatively.
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| 52. |
- Lundkvist, Annika, et al.
(författare)
-
Motor function after selective dorsal rhizotomy: a 10-year practice-based follow-up study.
- 2012
-
Ingår i: Developmental Medicine & Child Neurology. - : Wiley. - 0012-1622. ; 54:5, s. 429-435
-
Tidskriftsartikel (refereegranskat)abstract
- Aim The aim of this study was to explore changes in motor function up to 10 years after selective dorsal rhizotomy (SDR). Method The participants comprised 29 children (20 males, nine females) with bilateral spastic diplegia who were consecutively operated on at a median age of 4 years and 3 months and followed until a median age of 15 years. SDR was combined with physiotherapy and regular follow-up visits. The distribution of preoperative Gross Motor Function Classification System (GMFCS) levels was as follows: I, n=1; II, n=7; III, n=8; IV, n=12; and V, n=1. Muscle tone in hip flexors, hip adductors, knee flexors, and plantar flexors was assessed with the modified Ashworth scale, passive range of motion in hip abduction, popliteal angle, maximum knee extension, dorsiflexion of the foot was measured with a goniometer, and gross motor function was assessed using the Gross Motor Function Measure (GMFM-66). The results were compared with preoperative values, taking into account age at the time of SDR. Results After 10 years, muscle tone in hip flexors, hip adductors, knee flexors and plantar flexors was normalized in 19, 24, 13 and 23 participants respectively; mean change in passive range of motion ranged from -2.0° to 8.6°, and the mean increase in GMFM-66 was 10.6. Changes in GMFM-66 were associated with preoperative GMFCS level and GMFM-66 scores. Interpretation Children who underwent SDR and physiotherapy and were regularly followed up by an experienced team showed improved gross motor function for up to 10 years postoperatively.
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| 53. |
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| 54. |
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| 55. |
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| 56. |
- Lundkvist Josenby, Annika, et al.
(författare)
-
Longitudinal Construct Validity of the GMFM-88 Total Score and Goal Total Score and the GMFM-66 Score in a 5-Year Follow-up Study
- 2009
-
Ingår i: Physical Therapy. - : Oxford University Press (OUP). - 0031-9023 .- 1538-6724. ; 89:4, s. 342-350
-
Tidskriftsartikel (refereegranskat)abstract
- Background. The Gross Motor Function Measure (GMFM) is the instrument most commonly used to measure gross motor function in children with cerebral palsy (CP). Different scoring options have been developed, and their measurement properties have been assessed. Limited information is available regarding longitudinal construct validity. Objective. The objective of this research was to study the longitudinal construct validity of 3 scoring options: the 88-item GMFM (GMFM-88) total, the GMFM-88 goal total, and the 66-item GMFM (GMFM-66). Design. A clinical measurement design was used in this study. Methods. Forty-one children with CP diplegia who were undergoing selective dorsal rhizotomy (SDR) were monitored with the GMFM for 5 years. The mean age at SDR was 4.4 years (range=2.5-6.6). Two subgroups for gross motor function before surgery were created according to the Gross Motor Function Classification System (GMFCS): GMFCS levels I to III and GMFCS levels IV and V. This study included results obtained before SDR and at 6, 12, and 18 months and 3 and 5 years after SDR. The effect size (ES) and the standardized response mean (SRM) were calculated. Results. At 6 months postoperatively, ES and SRM values were small (>= 0.5) for all GMFM scoring options. The GMFM-88 total and goal total scores showed large changes in ES values (range=0.8-0.9) and SRM values (range=0.9-1.3) at 12 months postoperatively, whereas the GMFM-66 scores showed lower ES values (range=0.3-0.4) and SRM values (range=0.7-0.8) for both subgroups. Later postoperatively, larger values for longitudinal construct validity were found. The ES and SRM values generally were lower for the GMFM-66 scores than for the GMFM-88 total and goal total scores. Limitations. All children underwent an extensive intervention, and changes in gross motor function were expected. Conclusion. All 3 scoring options showed large longitudinal construct validity in the long-term follow-up. The GMFM-88 total and goal total scores revealed large changes in gross motor function earlier postoperatively than the GMFM-66 scores.
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| 57. |
- Lundtoft, Christian, et al.
(författare)
-
Complement C4 copy number variation is linked to SSA/Ro and SSB/La autoantibodies in systemic inflammatory autoimmune diseases.
- 2022
-
Ingår i: Arthritis & rheumatology (Hoboken, N.J.). - : Wiley. - 2326-5205 .- 2326-5191. ; 74:8, s. 1440-1450
-
Tidskriftsartikel (refereegranskat)abstract
- Copy number variation of the C4 complement components, C4A and C4B, has been associated with systemic inflammatory autoimmune diseases. We asked if C4 copy number variation is connected to the autoimmune repertoire in systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS) or myositis.Using targeted DNA sequencing, we determined the copy number and genetic variants of C4 in 2,290 well-characterised Scandinavian patients with SLE, pSS or myositis, and 1,251 healthy controls.A prominent relationship was observed between C4A copy number and the presence of SSA/SSB autoantibodies, which was shared between the three diseases. The strongest association was detected for patients with autoantibodies against both SSA and SSB and 0 C4A copies when compared to healthy controls (OR=18.0; CI95% : 10.2-33.3), whereas a weaker association was seen for patients without SSA/SSB autoantibodies (OR=3.1; CI95% : 1.7-5.5). The copy number of C4 correlated positively with C4 plasma levels. Further, a common loss-of-function variant in C4A leading to reduced plasma C4 was more prevalent in SLE patients with a low copy number of C4A. Functionally, we showed that absence of C4A reduced the individuals' capacity to deposit C4b on immune complexes.We show that a low C4A copy number more strongly is associated with the autoantibody repertoire than with the clinically defined disease entities. These results may have implication for understanding the aetiopathogenetic mechanisms of systemic inflammatory autoimmune diseases, and for patient stratification when taking the genetic profile into account. This article is protected by copyright. All rights reserved.
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| 58. |
- Lundtoft, Christian, et al.
(författare)
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Strong Association of Combined Genetic Deficiencies in the Classical Complement Pathway With Risk of Systemic Lupus Erythematosus and Primary Sjogren's Syndrome
- 2022
-
Ingår i: Arthritis & Rheumatology. - : Wiley. - 2326-5191 .- 2326-5205. ; 74:11, s. 1842-1850
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Tidskriftsartikel (refereegranskat)abstract
- Objective Complete genetic deficiency of the complement component C2 is a strong risk factor for monogenic systemic lupus erythematosus (SLE), but whether heterozygous C2 deficiency adds to the risk of SLE or primary Sjogren's syndrome (SS) has not been studied systematically. This study was undertaken to investigate potential associations of heterozygous C2 deficiency and C4 copy number variation with clinical manifestations in patients with SLE and patients with primary SS. Methods The presence of the common 28-bp C2 deletion rs9332736 and C4 copy number variation was examined in Scandinavian patients who had received a diagnosis of SLE (n = 958) or primary SS (n = 911) and in 2,262 healthy controls through the use of DNA sequencing. The concentration of complement proteins in plasma and classical complement function were analyzed in a subgroup of SLE patients. Results Heterozygous C2 deficiency-when present in combination with a low C4A copy number-substantially increased the risk of SLE (odds ratio [OR] 10.2 [95% confidence interval (95% CI) 3.5-37.0]) and the risk of primary SS (OR 13.0 [95% CI 4.5-48.4]) when compared to individuals with 2 C4A copies and normal C2. For patients heterozygous for rs9332736 with 1 C4A copy, the median age at diagnosis was 7 years earlier in patients with SLE and 12 years earlier in patients with primary SS when compared to patients with normal C2. Reduced C2 levels in plasma (P = 2 x 10(-9)) and impaired function of the classical complement pathway (P = 0.03) were detected in SLE patients with heterozygous C2 deficiency. Finally, in a primary SS patient homozygous for C2 deficiency, we observed low levels of anti-Scl-70, which suggests a risk of developing systemic sclerosis or potential overlap between primary SS and other systemic autoimmune diseases. Conclusion We demonstrate that a genetic pattern involving partial deficiencies of C2 and C4A in the classical complement pathway is a strong risk factor for SLE and for primary SS. Our results emphasize the central role of the complement system in the pathogenesis of both SLE and primary SS.
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| 59. |
- Magnusson, Charlotte, et al.
(författare)
-
Användarmedverkan – att delta i designprocesser
- 2015
-
Ingår i: Att arbeta med delaktighet inom habilitering. - 9789144107547
-
Bokkapitel (refereegranskat)abstract
- Kopplingen mellan design, hållbarhet och delaktighet har kommit att bli allt starkare i samhället. Till stor del beror det på att större förhoppningar ställs till arkitektur, form och design för att uppnå ökad sammanhållning, inkludering och tillgänglighet för alla människor i samhället (Kulturdepartementet 2015). Genom att ratificera FN:s konvention om rättigheter för personer med funktionsnedsättning (prop. 2008/09:28) har Sverige uttryckt en vilja att bygga ett samhälle på inkluderande, universellt utformade produkter, varor och tjänster. En sådan strävan innebär att miljöer och produkter utformas så att de fungerar för så många som möjligt utan att särskilda anpassningar krävs. Design anses alltmer som en samhällsförändrande kraft som har potential att skapa nya förutsättningar för människor att kunna ta del i och bidra till samhället (Kulturdepartementet 2015). I samband med denna utveckling är det naturligt att fokusera på hur de designprocesser som leder fram till nya produkter, tjänster och miljöer kan göras mer inkluderande och öppnas upp för påverkan. I detta kapitel presenteras exempel på hur man kan arbeta med design tillsammans med dem som ska använda den, samt vad ett sådant arbetssätt kan ge.
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| 60. |
- Mauritzson-Sandberg, Eva, et al.
(författare)
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ICT - the solution of communication hurdles in the modern family?
- 2004
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Ingår i: Human Perspectives in the Internet Society. - Southamton : WIT Press.
-
Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- The daily life situation for the family has changed drastically over the last few decades and during the same period of time the development within the area of information and communication technology (ICT) has, more or less, exploded. In this paper the two different development curves are combined in order to study the impact of new ICT applications on the daily life situation of the families of today. The two studies presented in this paper focuses primarily on the communication within families with children. The first study aims at mapping the patterns of communication within targeted families and their attitudes to, and need for, different ICT applications. In the second, trial set-ups of different ICT applications are evaluated. The results show that, although the families were assessing themselves as posi...
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| 61. |
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| 62. |
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| 63. |
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| 64. |
- Nordmark, Eva, et al.
(författare)
-
Cerebral pares
- 2006
-
Ingår i: Din barnläkare.
-
Bokkapitel (populärvet., debatt m.m.)
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| 65. |
- Nordmark, Eva, et al.
(författare)
-
Comparison of the Gross Motor Function Measure and Paediatric Evaluation of Disability Inventory in assessing motor function in children undergoing selective dorsal rhizotomy
- 2000
-
Ingår i: Developmental Medicine and Child Neurology. - : Wiley. - 0012-1622 .- 1469-8749. ; 42:4, s. 245-252
-
Tidskriftsartikel (refereegranskat)abstract
- This study was designed to compare assessment with the functional outcome measures Gross Motor Function Measure (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) over time, in children with cerebral palsy (CP) undergoing selective dorsal rhizotomy combined with individualised physiotherapeutic interventions. Using the Gross Motor Function Classification System (GMFCS), 18 children with spastic diplegia were divided into two groups according to age-related severity of motor function impairment. Data were collected preoperatively, and at 6 and la months postoperatively. Both instruments were sensitive to changes in function over time in the series as a whole and in the group with milder impairment, although the PEDI detected significant changes earlier. In the group with more severe impairment, changes in function were detected only with the PEDI, not with the GMFM. Thus, the instruments are to be considered complementary tests, because they measure different aspects of function.
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| 66. |
- Nordmark, Eva, et al.
(författare)
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Development of lower limb range of motion from early childhood to adolescence in cerebral palsy: a population-based study
- 2009
-
Ingår i: BMC Medicine. - : Springer Science and Business Media LLC. - 1741-7015. ; 7
-
Tidskriftsartikel (refereegranskat)abstract
- Background: The decreasing range of joint motion caused by insufficient muscle length is a common problem in children with cerebral palsy (CP), often worsening with age. In 1994 a CP register and health care programme for children with CP was initiated in southern Sweden. The aim of this study was to analyse the development of the passive range of motion (ROM) in the lower limbs during all the growth periods in relation to gross motor function and CP subtype in the total population of children with CP. Methods: In total, 359 children with CP born during 1990-1999, living in the southernmost part of Sweden in the year during which they reached their third birthday and still living in the area in the year of their seventh birthday were analysed. The programme includes a continuous standardized follow-up with goniometric measurements of ROM in the lower limbs. The assessments are made by each child's local physiotherapist twice a year until 6 years of age, then once a year. In total, 5075 assessments from the CPUP database from 1994 to 1 January 2007 were analysed. Results: The study showed a decreasing mean range of motion over the period 2-14 years of age in all joints or muscles measured. The development of ROM varied according to GMFCS level and CP subtype. Conclusion: We found a decreasing ROM in children with CP from 2-14 years of age. This information is important for both the treatment and follow-up planning of the individual child as well as for the planning of health care programmes for all children with CP.
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| 67. |
- Nordmark, Eva, et al.
(författare)
-
Hur blir delaktighet en helhet för barn och unga?
- 2015
-
Ingår i: Att arbeta med delaktighet inom habilitering. - Lund : Studentlitteratur AB. - 9789144107547 ; , s. 33-45
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Delaktighet har kommit att bli ett samlingsbegrepp för hur samhället ska kunna möta alla människors röster och perspektiv. Inte minst inom habiliteringens olika verksamheter är det idag ett centralt begrepp. Denna antologi tar sin utgångspunkt i ett mångfacetterat habiliteringsperspektiv och presenterar nya synsätt på hur delaktighet kan förstås, hur det praktiskt används, men också hur det kan problematiseras utifrån ett flervetenskapligt och tvärprofessionellt perspektiv. Kapitlen har ett praktiknära perspektiv och presenterar forskning gjord i nära samarbete med verksamhetsområden inom habilitering. För både yrkesverksamma, studerande och forskare reser antologin ett antal centrala frågor: Vad menas med delaktighet och på vilket sätt kan jag skapa delaktighet för andra människor? Är jag beredd att dela mitt inflytande och ansvar? Men också frågor om vem det är som ska bestämma vad som är en tillräcklig delaktighet, när den inte är tillräcklig och för vem denna delaktighet är tänkt? Med andra ord, hur kan delaktighet bli på riktigt? Detta är frågor som inte alltid har ett självklart svar men som är viktiga att diskutera. Det är också frågor som synliggör den spännvidd det finns i begreppet delaktighet och som gör det både lockande och spännande att arbeta med. Att arbeta med delaktighet inom habilitering vänder sig till universitets- och högskolestudenter som fokuserar på habilitering och delaktighet i olika typer av utbildningar. Även yrkesverksamma inom exempelvis habilitering och rehabilitering liksom samhällsaktörer inom området får en värdefull introduktion till tvärprofessionella och flervetenskapliga perspektiv på delaktighet.
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| 68. |
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| 69. |
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| 70. |
- Nordmark, Eva-Lisa, 1975-
(författare)
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Structural and Interaction Studies of Bacterial Polysaccharides by NMR Spectroscopy
- 2004
-
Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- An introduction to bacterial polysaccharides and the methods for structural determination are described in the first two parts of the thesis.In a structural elucidation of bacterial polysaccharides NMR experiments are important as is component analysis. A short description of immunochemical methods such as enzyme immunoassays is included. Two NMR techniques used for interaction studies, trNOE and STD NMR, are also discussed. The third part of the thesis discusses and summarizes the results from the included papers. The structures of the exopolysaccharides produced by two lactic acid bacteria are determined by one- and two dimensional NMR experiments. One is a heteropolysaccharide produced by Streptococcus thermophilus and the other a homopolysaccharide produced by Propionibacterium freudenreichii. The structure of an acidic polysaccharide from a marine bacterium with two serine residues in the repeating unit is also investigated. The structural and immunological relationship between two O-antigenic polysaccharides from Escherichia coli strain 180/C3 and O5 is discussed and investigated. Finally, interaction studies of an octasaccharide derived from the Salmonella enteritidis O-antigen and a bacteriophage are described which were performed with NMR experiments.
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| 71. |
- Nordmark, Eva-Lisa, et al.
(författare)
-
Structural studies of an exopolysaccharide produced by Streptococcus thermophilus THS
- 2005
-
Ingår i: Biomacromolecules. - : American Chemical Society (ACS). - 1525-7797 .- 1526-4602. ; 6:1, s. 105-108
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Tidskriftsartikel (refereegranskat)abstract
- The structure of an extracellular polysaccharide (EPS) from Streptococcus thermophilus THS has been determined. A combination of component analysis, methylation analysis and NMR spectroscopy shows that the polysaccharide is composed of pentasaccharide repeating units. Sequential information was obtained by two-dimensional 1H,1H−NOESY and 1H,13C−HMBC NMR experiments. NMR data indicate different mobility within the EPS with a stiffer backbone and a more flexible side-chain.
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| 72. |
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| 73. |
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| 74. |
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| 75. |
- Nordmark, Eva, et al.
(författare)
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Long-term outcomes five years after selective dorsal rhizotomy
- 2008
-
Ingår i: BMC Pediatrics. - : Springer Science and Business Media LLC. - 1471-2431. ; 8
-
Tidskriftsartikel (refereegranskat)abstract
- Background: Selective dorsal rhizotomy (SDR) is a well accepted neurosurgical procedure performed for the relief of spasticity interfering with motor function in children with spastic cerebral palsy (CP). The goal is to improve function, but long-term outcome studies are rare. The aims of this study were to evaluate long-term functional outcomes, safety and side effects during five postoperative years in all children with diplegia undergoing SDR combined with physiotherapy. Methods: This study group consisted of 35 children, consecutively operated, with spastic diplegia, of which 26 were Gross Motor Function Classification System (GMFCS) levels III-V. Mean age was 4.5 years (range 2.5-6.6). They were all assessed by the same multidisciplinary team at pre- and at 6, 12, 18 months, 3 and 5 years postoperatively. Clinical and demographic data, complications and number of rootlets cut were prospectively registered. Deep tendon reflexes and muscle tone were examined, the latter graded with the modified Ashworth scale. Passive range of motion (PROM) was measured with a goniometer. Motor function was classified according to the GMFCS and measured with the Gross Motor Function Measure (GMFM-88) and derived into GMFM-66. Parent's opinions about the children's performance of skills and activities and the amount of caregiver assistance were measured with Pediatric Evaluation Disability Inventory (PEDI). Results: The mean proportion of rootlets cut in S2-L2 was 40%. Muscle tone was immediately reduced in adductors, hamstrings and dorsiflexors (p<0.001) with no recurrence of spasticity over the 5 years. For GMFCS-subgroups I-II, III and IV-V significant improvements during the five years were seen in PROM for hip abduction, popliteal angle and ankle dorsiflexion (p=0.001), capacity of gross motor function (GMFM) (p=0.001), performance of functional skills and independence in self-care and mobility (PEDI) (p=0.001). Conclusion: SDR is a safe and effective method for reducing spasticity permanently without major negative side effects. In combination with physiotherapy, in a group of carefully selected and systematically followed young children with spastic diplegia, it provides lasting functional benefits over a period of at least five years postoperatively.
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| 76. |
- Nordmark, Eva
(författare)
-
Measurements of Function in Children with Cerebral Palsy
- 2000
-
Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Cerebral palsy (CP) is a term for a group of conditions associated with motor disabilities, that are very heterogeneous, i.e. symptoms, severity, associated impairments, functional outcome and needs. The overall purpose of this thesis was to study the prevalence and gross motor function and to evaluate and adapt measurements in children with CP. The prevalence in Southern Sweden was 2.4 per 1000 children. Children born abroad had a three times higher prevalence and were more often severely disabled. All 167 children were classified with the Gross Motor Function Classification System (GMFCS). This showed that 59% of the children were mildly disabled (levels I and II), 14% moderately disabled (level III) and 27% severely disabled (levels IV and V). Significant differences between GMFCS levels and subgroups of diagnosis, aetiology, intellectual capacity, epilepsy and visual impairment were found. Severe motor disability was often combined with associated impairments such as mental retardation, epilepsy and visual impairment. Inter- and intra-rater reliability of the Gross Motor Function Measure (GMFM) was found to be satisfying, even with untrained testers. The Pediatric Evaluation of Disability Inventory (PEDI) was investigated to ascertain the content, relevance and applicability in Sweden. A strong correlation between the PEDI results obtained for non-disabled Swedish children and the corresponding American data was found. This suggests that the American normative data are appropriate for reference purposes in Sweden. The GMFM and PEDI were compared regarding responsiveness in children with CP after Selective Dorsal Rhizotomy (SDR). Both GMFM and PEDI were sensitive to changes in function over time, for the series as a whole and in the group with mild to moderate disability, although the PEDI detected significant changes earlier. In the group with more severe disability, changes were detected only with the PEDI, not with the GMFM. The GMFM and PEDI are considered to be complementary tools as they measure different aspects of function. The usefulness of the Wartenberg pendulum test was investigated in very young children with spastic diplegia undergoing SDR.The pendulum test, combined with EMG, was shown to be an objective and sensitive method for quantifying spasticity in knee extensor muscles at ages down to 2.5 years. Children with spasticity displayed significantly lower values than healthy children. All the pendulum parameters improved after SDR and became similar to those of the healthy children. Swing time was the most reliable and sensitive variable. The R2 ratio showed a correlation with the quadriceps reflex. The relative swing time was the only parameter showing a correlation with measurements for function. Standardised measurements should be used as a part of the clinical decision-making process and for evaluation. They have to be multidimensional to address the variation of dysfunction at each level of the disablement process.
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| 77. |
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| 78. |
- Nordmark, Eva, et al.
(författare)
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Wartenberg pendulum test: objective quantification of muscle tone in children with spastic diplegia undergoing selective dorsal rhizotomy.
- 2002
-
Ingår i: Developmental Medicine & Child Neurology. - : Wiley. - 0012-1622 .- 1469-8749. ; 44:1, s. 26-33
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Tidskriftsartikel (refereegranskat)abstract
- The aim of this study was to investigate the reliability and sensitivity of the Wartenberg pendulum test for quantification of muscle tone in young children with spastic diplegia undergoing selective dorsal rhizotomy (SDR). Fourteen nondisabled children (mean age of 5.5 years, age range 2.3 to 8.8 years, one female and one male in each year) were tested twice. Twenty children with spastic diplegia (12 males, eight females; mean age of 4.3 years, age range 2.5 to 6.3 years) consecutively selected for SDR, were assessed before and 6 months after SDR. Parameters of the pendulum test: R2, R1, maximal velocity, and swing time were correlated with clinical assessments for spasticity (modified Ashworth scale, quadriceps reflex) and measurements of gross motor function: the Gross Motor Function Classification System and the Gross Motor Function Measure. The Wartenberg pendulum test was found to be an objective and sensitive method for quantifying spasticity in knee extensor muscles in children as young as 2.5 years old. The method was responsive to changes after SDR. The only correlation with clinical measurements of spasticity was between the R2 ratio and the quadriceps reflex. Swing time was the most reliable and sensitive variable; it showed a weak correlation with measurements for gross motor function.
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| 79. |
- Nordmark, Gunnel, et al.
(författare)
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Association of EBF1, FAM167A(C8orf13)-BLK and TNFSF4 gene variants with primary Sjögren's syndrome
- 2011
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Ingår i: Genes and Immunity. - : Springer Science and Business Media LLC. - 1466-4879 .- 1476-5470. ; 12:2, s. 100-109
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Tidskriftsartikel (refereegranskat)abstract
- We performed a candidate gene association study in 540 patients with primary Sjögren's Syndrome (SS) from Sweden (n=344) and Norway (n=196) and 532 controls (n=319 Swedish, n=213 Norwegian). A total of 1139 single-nucleotide polymorphisms (SNPs) in 84 genes were analyzed. In the meta-analysis of the Swedish and Norwegian cohorts, we found high signals for association between primary SS and SNPs in three gene loci, not previously associated with primary SS. These are the early B-cell factor 1 (EBF1) gene, P=9.9 × 10−5, OR 1.68, the family with sequence similarity 167 member A–B-lymphoid tyrosine kinase (FAM167A–BLK) locus, P=4.7 × 10−4, OR 1.37 and the tumor necrosis factor superfamily (TNFSF4=Ox40L) gene, P=7.4 × 10−4, OR 1.34. We also confirmed the association between primary SS and the IRF5/TNPO3 locus and the STAT4 gene. We found no association between the SNPs in these five genes and the presence of anti-SSA/anti-SSB antibodies. EBF1, BLK and TNFSF4 are all involved in B-cell differentiation and activation, and we conclude that polymorphisms in several susceptibility genes in the immune system contribute to the pathogenesis of primary SS.
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| 80. |
- Nordmark, Marie, 1967-
(författare)
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Digitalt skrivande i gymnasieskolans svenskundervisning : en ämnesdidaktisk studie av skrivprocessen
- 2014
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- The aim of this thesis is to investigate the writing process in the teaching of the subject of Swedish at upper secondary school. This study analyses the relations between the pupils’ writing and the school environment in which the writing takes place and how the pupils position themselves and others in relation to their use of digital artefacts and norms in the classroom. The empirical material has been created in three classes at two different schools. The data consists of participant observations of 42 lessons, all of which were video recorded using two cameras, and audio recorded semi-structured interviews with 24 pupils and 3 teachers. Theoretically, the study is based on sociocultural perspectives on literacy and learning and a multimodal social semiotic understanding of meaning-making based on an interest in the use of resources that constitute meaning in the social environment. From an ecological perspective, writing is examined as discourses in which the participants and the environment interact. Analytic concepts are used by inspiration from Kress et al (2005) and Smidt (2002). The figure “Writing roles in fields of tension” has been constructed to illustrate the students’ writing roles and positioning's in the empirical material. The results show that the shift from paper and pen to computer and screen means more than a shift change in the use of artefacts. The teaching of digital writing has a point of departure as a project in communication. In multimodal environments, pupils are often left without access to a teacher due to the layout of the room. This leads to positioning in roles, such as help seeker and helper. The classrooms are characterised by the constant presence of social media and its demands on students’ attention. Earlier generations of writing processes emphasised the importance of prewriting, drafting and revision in stages. In the digital writing process these stages are lacking. In this context, the digital writing process can be understood as a “fourth generation process” consisting of writing, saving and sending. The fourth generation of writing process stresses on the writing at a micro- rather than macro level. The word processing functions of spelling and grammar offer clickable solutions to problems, but cannot be considered as tools for learning. In the digital classroom pupils are vulnerable, left to their own resources and have difficulties in handling complex assignments.
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| 81. |
- Nordmark, Sofi
(författare)
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Hindrances and Feasibilities that Affect Discharge Planning : Perspectives Before and After the Development and Testing of ICT Solutions
- 2016
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Deficits in collaboration and information exchange during patient discharge from the hospital can cause negative consequences for the patient, such as delayed discharge, readmission, inadequate post-discharge care, and reduced quality of life. Information and communication technology (ICT) can contribute to easing and securing information exchange and collaboration at care transitions from one care provider to another. The overall aim of this doctoral thesis was to gain broader knowledge about the discharge planning process by exploring the experiences of the involved personnel both before and after the development and testing of ICT solutions.Studies I, II and III were conducted before the development and testing of ICT solutions. In study I, the experiences and views of registered nurses (RNs) and district nurses (DNs) regarding information exchange during the discharge planning process (DPP) for patients who required primary healthcare after discharge from hospital were probed. Data were collected through a web based questionnaire and were analysed with descriptive statistics, chi-squared test and qualitative text analysis. In study II, the experiences and views of DNs and homecare organisers regarding the DPP were explored. Individual interviews were conducted with nine DNs and five homecare organisers working in homecare. Data were analysed via qualitative content analysis using an inductive approach. In study III, the DPP was evaluated. Qualitative data from studies I and II together with data the development process and 12 interviews with RNs in hospital care were analysed using Normalisation Process Theory. In study IV, RNs’, DNs’ and homecare organisers’ experiences with an agile development process that included ICT testing were explored. An evaluation form was used to collect data on the experiences of RNs and DNs using videoconference technology. Data were analysed with descriptive statistics. Seven focus group interviews were also performed with 11 RNs, nine DNs and four homecare organisers after the development and testing of ICT. Data were analysed using qualitative content analysis. The results showed significant differences in perceived information exchange (I). Hindrances and feasibilities at the organisational, group and individual levels all affected the workflow. Demands, workload, time, collaboration, and engagement together with knowledge and professional confidence were factors that influenced the DPP (II). The results revealed that the previously DPP was not normalised or embedded within daily work before the development project began (III). RNs, DNs and homecare organisers had reached consensus on what the process was about (coherence) and how they evaluated the process (reflexive monitoring) but not on who performed the process (cognitive participation) and how it was performed (collective action). By integrating an agile development process with the results obtained from studies I, II and III, ICT solutions aiming to support the DPP were tested in study IV. Study IV revealed that the time and timing both at the individual, group, and organisational levels and between those levels was essential for the development and adoption of new processes and products among caregivers. Keywords: discharge planning process, collaboration, information exchange, ICT, experiences, nurses, homecare organisers, qualitative content analysis, descriptive statistics
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| 82. |
- Sepulveda, Jorge I. Ramirez, et al.
(författare)
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Long-term follow-up in primary Sjögren's syndrome reveals differences in clinical presentation between female and male patients
- 2017
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Ingår i: Biology of Sex Differences. - : BioMed Central. - 2042-6410. ; 8
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Tidskriftsartikel (refereegranskat)abstract
- Background: Despite men being less prone to develop autoimmune diseases, male sex has been associated with a more severe disease course in several systemic autoimmune diseases. In the present study, we aimed to investigate differences in the clinical presentation of primary Sjogren's syndrome (pSS) between the sexes and establish whether male sex is associated with a more severe form of long-term pSS. Methods: Our study population included 967 patients with pSS (899 females and 68 males) from Scandinavian clinical centers. The mean follow-up time (years) was 8.8 +/- 7.6 for women and 8.5 +/- 6.2 for men (ns). Clinical data including serological and hematological parameters and glandular and extraglandular manifestations were compared between men and women. Results: Male patient serology was characterized by more frequent positivity for anti-Ro/SSA and anti-La/SSB (p = 0. 02), and ANA (p = 0.02). Further, men with pSS were more frequently diagnosed with interstitial lung disease (p = 0. 008), lymphadenopathy (p = 0.04) and lymphoma (p = 0.007). Conversely, concomitant hypothyroidism was more common among female patients (p = 0.009). Conclusions: We observe enhanced serological responses and higher frequencies of lymphoma-related extraglandular manifestations in men with pSS. Notably, lymphoma itself was also significantly more common in men. These observations may reflect an aggravated immune activation and a more severe pathophysiological state in male patients with pSS and indicate a personalized managing of the disease due to the influence of the sex of patients with pSS.
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| 83. |
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| 84. |
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| 85. |
- Stahlhut, Michelle, et al.
(författare)
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Building the repertoire of measures of walking in Rett syndrome
- 2017
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Ingår i: Disability and Rehabilitation. - : Informa UK Limited. - 0963-8288 .- 1464-5165. ; 39:19, s. 1926-1931
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Tidskriftsartikel (refereegranskat)abstract
- Background: The repertoire of measures of walking in Rett syndrome is limited. This study aimed to determine measurement properties of a modified two-minute walk test (2MWT) and a modified Rett syndrome-specific functional mobility scale (FMS-RS) in Rett syndrome. Methods: Forty-two girls and women with Rett syndrome (median 18.4 years, range 2.4–60.9 years) were assessed for clinical severity, gross motor skills, and mobility. To measure walking capacity, 27 of this group completed a 2MWT twice on two different assessment days. To assess walking performance, the FMS-RS was administered to the total sample of parents (n = 42) on two occasions approximately one week apart. Results: There were negative correlations between clinical severity and 2MWT (r = −0.48) and FMS-RS (r = −0.60–0.66). There were positive correlations between gross motor skills and mobility and 2MWT (r = 0.51, 0.43) and FMS-RS (r = 0.71–0.93, 0.74–0.94), respectively. Test–retest reliability for the 2MWT was good with high intraday and interday correlations (ICC = 0.86–0.98). For the 2MWT, the standard error of measurement was 13.8 m and we would be 95% confident that changes greater than 38 m would be greater than within subject error. There was good test–retest reliability for all three distances on the FMS-RS (ICC = 0.94–0.99). Conclusions: Walking capacity as measured by the 2MWT showed expected but limited relationships with measures of different constructs, providing some support for concurrent validity. Walking performance as measured with the FMS-RS was more strongly consistent with other clinical measures supporting its concurrent validity. Test–retest reliability was good for both the FMS-RS and the 2MWT. Therefore, these measures have the potential to be used in clinical practice and research. Implications for RehabilitationWalking is one of the commonest daily physical activities in ambulant girls and women with RTT. Comprehensive knowledge about the walking abilities in this population is limited.Evidence of validity and test–retest reliability have been demonstrated for the modified two-minute walk test (2MWT) and the Rett syndrome-specific functional mobility scale (FMS-RS).The 2MWT and FMS-RS offer detailed information of the capacity and performance of walking, respectively, in girls and women with RTT
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| 86. |
- Stahlhut, Michelle, et al.
(författare)
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Facilitators and Barriers of Participation in “Uptime” Activities in Girls and Women With Rett Syndrome : Perspectives From Parents and Professionals
- 2019
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Ingår i: Qualitative Health Research. - : SAGE Publications. - 1049-7323 .- 1552-7557. ; 29:4, s. 609-619
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Tidskriftsartikel (refereegranskat)abstract
- Rett syndrome (RTT) is a rare neurodevelopmental disorder usually affecting females. It is associated with intellectual and multiple disabilities leading to a high level of dependency in all aspects of daily living including participation in physical activities. This study explored facilitators and barriers to “uptime” (non-sedentary) activities in Danish girls and women with RTT as perceived by parents and professionals using focus groups. Through thematic analysis, one central theme emerged: a constant balance to do the best thing for the girl or woman. Within the central theme, five subthemes of facilitators and barriers were identified relating to the individual and the physical, organizational, social, and attitudinal environments. Environmental barriers can be reduced through policy and management-level changes in health promotion and strong advocacy of physical activity by health professionals. Targeting both facilitators and barriers of “uptime” activities enables the planning and implementing of health-promoting interventions in individuals with RTT.
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| 87. |
- Stahlhut, Michelle, et al.
(författare)
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Feasibility and Effectiveness of an Individualized 12-Week "Uptime" Participation (U-PART) Intervention in Girls and Women With Rett Syndrome
- 2020
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Ingår i: Physical Therapy. - : Oxford University Press (OUP). - 0031-9023 .- 1538-6724. ; 100:1, s. 168-179
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Girls and women with Rett Syndrome (RTT) have low levels of daily physical activity and high levels of sedentary time. Reducing sedentary time and enhancing "uptime" activities, such as standing and walking, could be an important focus for interventions to address long-term health and quality of life in RTT. OBJECTIVE: The aim of the study was to evaluate the feasibility and health-related effects of an individualized 12-week uptime participation (U-PART) intervention in girls and women with RTT. DESIGN: The study used a single-group pretest-posttest design with 4 assessments (2 baseline, postintervention, and follow-up). METHODS: A participation-based intervention employing a whole-day approach was used. During a 12-week intervention period, individualized programs focused on participation in enjoyable uptime activities in home, school/day center, and community settings. Feasibility was assessed with a study-specific questionnaire. Primary outcome measures were sedentary time and daily step count. Secondary outcomes were gross motor skills, walking capacity, quality of life, and goal attainment scaling. RESULTS: Fourteen girls and women who were 5 to 48 years old and had RTT participated. The U-PART intervention was perceived as feasible by caregivers. Similar scores were observed at baseline assessments in all outcomes. Positive effects with small to medium effect sizes (0.27-0.54) were seen in sedentary time (- 4%), daily step count (+ 689 steps/d), walking capacity (+ 18.8 m), quality of life (+ 2.75 points), and goal attainment scaling after the intervention. Positive effects were maintained in sedentary time (- 3.2%) and walking capacity (+ 12.1 m) at short-term follow-up. LIMITATIONS: This study was limited by the lack of a control group. However, participants acted as their own control, and the stable baseline period partially mitigated this issue. CONCLUSIONS: The U-PART intervention was found to be feasible and effective in the short term in girls and women with RTT.
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| 88. |
- Stahlhut, Michelle, et al.
(författare)
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Patterns of sedentary time and ambulatory physical activity in a Danish population of girls and women with Rett syndrome
- 2019
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Ingår i: Disability and Rehabilitation. - : Informa UK Limited. - 0963-8288 .- 1464-5165. ; 41:2, s. 133-141
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Tidskriftsartikel (refereegranskat)abstract
- Background: Rett syndrome (RTT) is a rare neurodevelopmental disorder leading to multiple disabilities and high dependency on caregivers. This study aimed to: (1) describe the patterns of sedentary time and daily steps and (2) identify the association of individual and environmental characteristics with sedentary time. Methods: All Danish females with RTT older than 5 years of age and with a MECP2 mutation were invited to participate. The activPAL and StepWatch Activity Monitor (SAM) were worn by participants for at least four days. Sedentary time and step counts were plotted by time to examine daily activity patterns. Associations between sedentary time and individual and environmental covariates were assessed with linear regression models. Results: The median (interquartile range) age of participants was 22.0 (14.3–36.5) years. On average 83.3% (standard deviation 13.9%) of waking hours were spent in sedentary behaviours (n = 48) and the median (interquartile range) daily step count was 5128 (2829–7704) (n = 28). Females older than 33.5 years, and those unable to walk independently were more sedentary. Conclusions: This study demonstrated high levels of sedentary time and low daily step counts in a Danish population of females with RTT. Advancing age and lower walking skills were associated with higher levels of sedentary time.Implications for RehabilitationSedentary lifestyles in individuals with disabilities have a negative impact on health and quality of life.High levels of sedentary time and low daily step counts were demonstrated in a Danish population of females with Rett syndrome.Advancing age and inability to walk independently were strongly associated with higher levels of sedentary time in females with Rett syndrome.Understanding patterns of sedentary behaviour and physical activity can aid health care professionals in developing health-promoting physical activity interventions.
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| 89. |
- Theander, Elke, et al.
(författare)
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Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjogren's syndrome
- 2011
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Ingår i: Annals of the Rheumatic Diseases. - : BMJ. - 0003-4967 .- 1468-2060. ; 70:8, s. 1363-1368
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE:The development of non-Hodgkin's lymphoma (NHL) confers a high risk of mortality in primary Sjögren's syndrome (pSS) patients, but the sensitivity and specificity of proposed lymphoma predictors are insufficient for practical use. The performance of lymphoid organisation in the form of germinal centre (GC)-like lesions was evaluated in labial salivary gland biopsies taken at pSS diagnosis as a potential lymphoma-predicting biomarker.METHODS:Labial salivary gland tissue biopsies available from two Swedish pSS research cohorts (n=175) were re-evaluated by light microscopy in a blind study in order to identify GC-like structures as a sign of ectopic lymphoid tissue formation and organisation. A linkage study was performed with the Swedish Cancer Registry for lymphoma identification. The risk of developing NHL in GC-positive patients in comparison with GC-negative patients was evaluated using Kaplan-Meier statistics and log-rank test. Associations between GC-like structures and clinical and/or laboratory disease markers were also determined using χ(2) or Fisher's exact tests.RESULTS:At diagnosis, 25% of pSS patients had GC-like structures in their salivary glands. Seven of the 175 patients studied (14% GC+ and 0.8% GC-) developed NHL during 1855 patient-years at risk, with a median onset of 7 years following the initial diagnostic salivary gland biopsy. Six of the seven patients had GC-like structures at diagnosis; the remaining patient was GC negative at the time of diagnosis (p=0.001).CONCLUSIONS:The detection of GC-like structures by light microscopy in pSS diagnostic salivary biopsies is proposed as a highly predictive and easy-to-obtain marker for NHL development. This allows for risk stratification of patients and the possibility to initiate preventive B-cell-directed therapy.
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| 90. |
- Thorlacius, Guðný Ella, et al.
(författare)
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Genetic and clinical basis for two distinct subtypes of primary Sjögren's syndrome
- 2021
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Ingår i: Rheumatology. - : Oxford University Press. - 1462-0324 .- 1462-0332. ; 60:2, s. 837-848
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Tidskriftsartikel (refereegranskat)abstract
- ObjectivesClinical presentation of primary Sjögren’s syndrome (pSS) varies considerably. A shortage of evidence-based objective markers hinders efficient drug development and most clinical trials have failed to reach primary endpoints.MethodsWe performed a multicentre study to identify patient subgroups based on clinical, immunological and genetic features. Targeted DNA sequencing of 1853 autoimmune-related loci was performed. After quality control, 918 patients with pSS, 1264 controls and 107 045 single nucleotide variants remained for analysis. Replication was performed in 177 patients with pSS and 7672 controls.ResultsWe found strong signals of association with pSS in the HLA region. Principal component analysis of clinical data distinguished two patient subgroups defined by the presence of SSA/SSB antibodies. We observed an unprecedented high risk of pSS for an association in the HLA-DQA1 locus of odds ratio 6.10 (95% CI: 4.93, 7.54, P=2.2×10−62) in the SSA/SSB-positive subgroup, while absent in the antibody negative group. Three independent signals within the MHC were observed. The two most significant variants in MHC class I and II respectively, identified patients with a higher risk of hypergammaglobulinaemia, leukopenia, anaemia, purpura, major salivary gland swelling and lymphadenopathy. Replication confirmed the association with both MHC class I and II signals confined to SSA/SSB antibody positive pSS.ConclusionTwo subgroups of patients with pSS with distinct clinical manifestations can be defined by the presence or absence of SSA/SSB antibodies and genetic markers in the HLA locus. These subgroups should be considered in clinical follow-up, drug development and trial outcomes, for the benefit of both subgroups.
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| 91. |
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| 92. |
- Uddenfeldt Wort, Ulrika, et al.
(författare)
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Fractures in children with cerebral palsy: a total population study.
- 2013
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Ingår i: Developmental Medicine & Child Neurology. - : Wiley. - 0012-1622. ; 55:9, s. 821-826
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Tidskriftsartikel (refereegranskat)abstract
- AIM: To analyse factors associated with fractures in children with cerebral palsy (CP) in different levels of Gross Motor Function Classification System (GMFCS). METHOD: This was an epidemiological retrospective study of a total population of 536 children (214 females, 322 males) with CP born between 1990 and 2005. CP type was unilateral spastic (n=159), bilateral spastic (n=225), ataxic (n=60), dyskinetic (n=80), and mixed type (n=12); 384 children were in Gross Motor Function Classification Scale (GMFCS) levels I-III and 152 children were in GMFCS levels IV-V. Data were collected for a 9-year period on sex, CP-type, GMFCS level, gastrostomy, height, weight, the use of a standing device, antiepileptic drug (AED) therapy, and fractures. RESULTS: The risk of fracture in the total population of children with CP was similar to that for typically developing children. The risk for fractures of those in GMFCS levels I-III was not significantly associated with any of the studied risk factors. The risk of fractures for those in GMFCS levels IV-V on AED therapy was a twofold increase (p=0.004). The risk for fractures without trauma in children with stunted growth (height for age <-3 SD) and those who did not use standing devices was significantly increased: adjusted incidence rate ratio (AIRR) 4.16 (p=0.011) and 3.66 (p=0.010) respectively. Results regarding gastrostomy feeding for those in GMFCS levels IV-V were conflicting: a gastrostomy was associated with a reduced risk of fractures with trauma, but with increased risk of fractures without trauma (AIRR 0.10, p=0.003 and 4.36, p=0.012) respectively. INTERPRETATION: Children in GMFCS levels I-III had a similar incidence and pattern for fractures as normally developing children. Those in GMFCS levels IV-V had stunted growth, often a sign of longstanding undernourishment, and were associated with an increased risk of fractures. Children using standing devices had a fourfold reduction of fractures without trauma. Regular loading exercises and early adequate nutritional intake could prevent fractures in severe CP.
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| 93. |
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| 94. |
- Urbina, Felipe, et al.
(författare)
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Structural elucidation of the O-antigenic polysaccharide from the enteroaggregative Escherichia coli strain 180/C3 and its immunochemical relationship with Escherichia coli O5 and O65
- 2005
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Ingår i: Carbohydrate Research. - : Elsevier Ltd. - 0008-6215 .- 1873-426X. ; 340:4, s. 645-650
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Tidskriftsartikel (refereegranskat)abstract
- The structure of the O-antigen polysaccharide (PS) from the enteroaggregative Escherichia coli strain 180/C3 has been determined. Sugar and methylation analysis together with 1H and 13C NMR spectroscopy were the main methods used. The PS is composed of tetrasaccharide repeating units with the following structure:→2)-β-d-Quip3NAc-(1→3)-β-d-Ribf-(1→4)-β-d-Galp-(1→3)-α-d-GalpNAc-(1→Analysis of NMR data indicates that the presented sequence of sugar residues also represents the biological repeating unit of the O-chain. The structure is closely related to that of O-antigen polysaccharide from E. coli O5 and partially to that of E. coli O65. The difference between the O-antigen from the 180/C3 strain and that of E. coli O5 is the linkage to the d-Quip3NAc residue, which in the latter strain is 4-O-substituted. The E. coli O65 O-antigen contains as part of its linear pentasaccharide repeating unit a similar structural element, namely →4)-β-d-GalpA-(1→3)-α-d-GlcpNAc-(1→2)-β-d-Quip3NAc-(1→, thereby indicating that a common epitope could be present for the two polysaccharides. Monospecific anti-E. coli O5 rabbit serum did not distinguish between the two positional isomeric structures neither in slide agglutination nor in an indirect enzyme immunoassay. The anti-O65 serum did react with both the 180/C3 and O5 LPS showing a partial cross-reactivity.
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| 95. |
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| 96. |
- Vasaitis, Lilian, et al.
(författare)
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Comparison of patients with and without pre-existing lymphoma at diagnosis of primary Sjögren's syndrome
- 2019
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Ingår i: Scandinavian Journal of Rheumatology. - : Informa UK Limited. - 0300-9742 .- 1502-7732. ; 48:3, s. 207-212
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: In the 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome (pSS), pre-existing lymphoma is not an exclusion criterion for pSS diagnosis, as in earlier criteria. We aimed to explore whether there are differences between pSS patients with and without pre-existing lymphoma at pSS diagnosis.METHOD: Patients with ICD-7-10 codes for Sjögren's syndrome (SS) and a diagnosis of malignant lymphoma before or after SS diagnosis were identified by linking the Swedish Patient Register 1964-2007 with the Cancer Register 1990-2007 (n = 224). Clinical data were collected from medical records. Lymphoma diagnoses were evaluated by tissue review. Characteristics of pSS patients with and without pre-existing lymphoma were compared.RESULTS: We identified 107 patients with pSS as the reason for an SS diagnosis code and a verified lymphoma. Of these, 18 (17%) had a pre-existing lymphoma at pSS diagnosis, defined as lymphoma diagnosed before or within 6 months of pSS diagnosis. Male gender (39% vs 10%, p = 0.006), enlarged lymph nodes during the pSS disease (61% vs 27%, p = 0.01), mucosa-associated lymphoid tissue (MALT) lymphoma (50% vs 22%, p = 0.02), and salivary gland lymphoma (61% vs 26%, p = 0.006) were more common in patients with a pre-existing lymphoma at pSS diagnosis. Other pSS characteristics were similar.CONCLUSION: In a substantial proportion of patients, particularly in men, pSS remains undiagnosed until after lymphoma diagnosis. The study highlights the importance of pSS investigation in patients with lymphoma, especially MALT lymphoma, in the salivary glands.
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| 97. |
- Vasaitis, Lilian, et al.
(författare)
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Population-based study of patients with primary Sjögren’s syndrome and lymphoma : lymphoma subtypes, clinical characteristics, and gender differences
- 2020
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Ingår i: Scandinavian Journal of Rheumatology. - : Informa UK Limited. - 0300-9742 .- 1502-7732. ; 49:3, s. 225-232
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To examine lymphoma subtypes, clinical characteristics, and gender differences in patients with primary Sjögren’s syndrome (pSS) and lymphoma in a population-based setting. Method: Patients with Sjögren’s syndrome and lymphoma diagnoses were identified by linkage of the Swedish Patient Register 1964–2007 with the Cancer Register 1990–2007. Clinical data were collected from medical records and lymphoma tissues were re-examined. The lymphoma subtype distribution was compared with the Swedish Lymphoma Register. Results: We identified 105 pSS patients with lymphoma. Diffuse large B-cell lymphoma (DLBCL) (32%) and marginal zone lymphoma [MZL including mucosa-associated lymphoid tissue (MALT) lymphoma] (31%) were the most common lymphoma subtypes. The proportion of DLBCL was not increased compared to the general population reference (32%, p = 1), in contrast to MZL (general population 5%, p < 0.0001). Compared to DLBCL, MALT lymphoma was diagnosed at a younger age (55 vs 67 years, p = 0.0001), and earlier after patient-reported sicca onset (7 vs 18 years, p = 0.0001) and pSS diagnosis (2 vs 9 years, p = 0.0005). Sixteen of the pSS-lymphoma cases were men (15%), twice the proportion in general pSS populations. Compared to women, men had a shorter median time from pSS diagnosis to lymphoma diagnosis (1 vs 8 years, p = 0.0003) and more often had lymphoma in the salivary glands (56% vs 29%, p = 0.04). Conclusion: DLBCL and MZL are common in pSS patients, but only MZL/MALT lymphoma occurs at an increased relative frequency in pSS compared to the general population. The study supports increased awareness of signs of lymphoma in men in the first years after pSS diagnosis.
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| 98. |
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| 99. |
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| 100. |
- Vasaitis, Lilian, et al.
(författare)
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Sporadic occurrence of non-diagnosed IgG4-related disease in lymphoma patients with a previous Sjögren's syndrome diagnosis.
- 2016
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Ingår i: Acta Oncologica. - 0284-186X .- 1651-226X. ; 55:9-10, s. 1139-1144
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disorder, which may affect many organs, and often comes to clinical attention due to tumor-like organ swelling or is identified incidentally by specific biopsy findings. Typical histopathology of IgG4-RD is lymphoplasmacytic infiltration rich in IgG4 + plasma cells (PCs), storiform fibrosis, and obliterative phlebitis. Patients with sicca symptoms can be misdiagnosed as primary Sjögren's syndrome (pSS) instead of IgG4-RD because of clinical and histopathological similarities. Moreover, an association with lymphoma development is described in both diseases. This study investigated signs of IgG4-RD in a population-based cohort of patients diagnosed with pSS complicated by lymphoma.METHODS: Patients with pSS and lymphoma diagnoses and available lymphoma specimens were identified by linkage with the Swedish Patient Register 1964-2007 and the Cancer Register 1990-2007 (n = 79). Clinical data and lymphomas were reviewed and the diagnoses evaluated. All lymphoma tissues and available minor salivary gland biopsies (n = 11) were immunostained for IgG4 + PCs and evaluated for other histopathological signs of IgG4-RD. In a case with specific findings of IgG4-RD, other available tissue specimens of the same patient were investigated for IgG4-RD.RESULTS: Only one patient of 79 (1.3%) had >10 IgG4 + PCs/high power field (HPF) in the lymphoma tissue, an unspecified low-grade B-cell lymphoma localized in the submandibular gland. This patient also had other histopathological features of IgG4-RD in the lymphoma and a surgical lung biopsy taken five years before lymphoma diagnosis and, therefore, fulfilled the criteria for IgG4-RD. Occasional IgG4 + PCs (<10/HPF) without signs of IgG4-RD were observed in another six lymphomas. No IgG4 + PCs were identified in the minor salivary gland biopsies.CONCLUSION: Histopathological findings of IgG4-RD may co-exist with low malignant B-cell lymphoma in patients with initially suspected pSS and may be associated with an underlying IgG4-RD.
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