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1.
  • Holmstrom, Gerd, et al. (författare)
  • Treatment for retinopathy of prematurity in Sweden 2008-2021: Reduced gestational age of treated infants and remaining differences in treatment type and recurrence rates between hospitals
  • 2024
  • Ingår i: ACTA OPHTHALMOLOGICA. - : John Wiley & Sons. - 1755-375X .- 1755-3768. ; 102:4, s. 401-408
  • Tidskriftsartikel (refereegranskat)abstract
    • PurposeThis study aimed to investigate various aspects of treatment for retinopathy of prematurity (ROP) in Sweden over the past 14 years, nationally and at a hospital level.MethodsData on screening and treatment for ROP in infants born in Sweden from 2008 to 2021 were extracted from the national ROP register, SWEDROP. During this period, Swedish screening guidelines were reduced from gestational age (GA) < 32 weeks to <31 weeks in 2012 and to <30 weeks in 2020.ResultsAltogether, 10 959 infants were screened and 600 infants treated for ROP during the study period. Parallel to changed guidelines, the number of screened infants decreased (p < 0.000) and the incidence of ROP and frequency of treatment increased (p < 0.001), while both remained similar in infants with a GA below 30 weeks. Among treated infants, GA and BW were reduced over the years (p < 0.001). Laser treatment (85.2% of primary treatments) became less common and anti-VEGF injections (13.6%) became more common over time (p < 0.001). Altogether 16 eyes were treated with the encircling band and 13 with vitrectomy. The total frequency of retreatment (32.7% of treated eyes) remained similar over time but was more common after primary anti-VEGF injection (67.7%) than laser treatment (27.2%). There were differences between the seven university hospitals regarding type of treatment and number of retreatments (p < 0.001).ConclusionThe frequency of treatment and retreatment for ROP remained similar over time, but the type of treatment changed and anti-VEGF injections became more common. Differences between treating hospitals emphasize the importance of centralizing the most severe cases.
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2.
  • Austeng, Dordi, et al. (författare)
  • Incidence of and risk factors for neonatal morbidity after active perinatal care : extremely preterm infants study in Sweden (EXPRESS)
  • 2010
  • Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 99:7, s. 978-992
  • Tidskriftsartikel (refereegranskat)abstract
    • Aims: The aim of this study was to determine the incidence of neonatal morbidity in extremely preterm infants and to identify associated risk factors. Methods: Population based study of infants born before 27 gestational weeks and admitted for neonatal intensive care in Sweden during 2004-2007. Results: Of 638 admitted infants, 141 died. Among these, life support was withdrawn in 55 infants because of anticipation of poor long-term outcome. Of 497 surviving infants, 10% developed severe intraventricular haemorrhage (IVH), 5.7% cystic periventricular leucomalacia (cPVL), 41% septicaemia and 5.8% necrotizing enterocolitis (NEC); 61% had patent ductus arteriosus (PDA) and 34% developed retinopathy of prematurity (ROP) stage >= 3. Eighty-five per cent needed mechanical ventilation and 25% developed severe bronchopulmonary dysplasia (BPD). Forty-seven per cent survived to one year of age without any severe IVH, cPVL, severe ROP, severe BPD or NEC. Tocolysis increased and prolonged mechanical ventilation decreased the chances of survival without these morbidities. Maternal smoking and higher gestational duration were associated with lower risk of severe ROP, whereas PDA and poor growth increased this risk. Conclusion: Half of the infants surviving extremely preterm birth suffered from severe neonatal morbidities. Studies on how to reduce these morbidities and on the long-term health of survivors are warranted.
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3.
  • Austeng, Dordi, et al. (författare)
  • Natural history of retinopathy of prematurity in infants born before 27 weeks' gestation in Sweden
  • 2010
  • Ingår i: Archives of ophthalmology (1960). - : American Medical Association. - 0003-9950. ; 128:10, s. 1289-1294
  • Tidskriftsartikel (refereegranskat)abstract
    • Objective: To investigate the natural history of retinopathy of prematurity (ROP) in 506 extremely preterm infants born before 27 weeks' gestation in Sweden during a 3-year period. Methods: A national population–based study was performed in Sweden from April 1, 2004, to March 31, 2007. According to the study protocol, initial eye examinations were to be performed at postnatal week 5, and examinations were repeated until the retina was completely vascularized or until criteria for treatment were met. The examinations were to be performed weekly, enabling study of the course and severity of ROP. In infants without ROP or with mild ROP without progression during the latest examinations, further examinations were performed weekly or every other week from postmenstrual age 35 weeks. Results: During the study, 368 infants (72.7%) developed ROP. Postmenstrual age at onset of ROP was significantly related to severity of ROP, even when controlling for gestational age (ie, the earlier the onset of ROP, the higher the risk of developing severe ROP). Site of onset of ROP was significantly related to gestational age at birth. The risk of nasal onset was almost doubled for every week of decrease in gestational age at birth. Nasal onset was associated with severe ROP, even after adjusting for gestational age at birth. Conclusion: This population-based study confirms results of the Cryotherapy for Retinopathy of Prematurity study and shows new correlations regarding time and site of onset of ROP, both of which are associated with disease severity.
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4.
  • Austeng, Dordi, et al. (författare)
  • Regional differences in screening for retinopathy of prematurity in infants born before 27 weeks of gestation in Sweden : the EXPRESS study
  • 2014
  • Ingår i: Acta Ophthalmologica. - : Wiley-Blackwell. - 1755-375X .- 1755-3768. ; 92:4, s. 311-315
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose: The primary aim was to analyse regional incidences of retinopathy of prematurity (ROP) and frequencies of treatment and their relation to perinatal risk factors during a 3-year period. A secondary aim was to study adherence to the study screening protocol in the different regions.Methods: A population-based study of neonatal morbidity in extremely preterm infants in Sweden (EXPRESS) was performed during 2004-2007. Screening for ROP was to start at postnatal age 5weeks and to continue weekly until the retina was completely vascularized or until regression of ROP. Logistic regression analyses were used for evaluation of differences in incidence of Any ROP, ROP 3 or more and ROP Type 1 between the seven regions of the country.Results: The regional incidence of ROP varied between 54% and 92% for Any ROP, between 25% and 43% for ROP stage 3 or more and between 8% and 23% of infants with ROP Type 1, all of whom were treated. There was no significant difference between the regions regarding ROP Type 1, even when adjusting for known risk factors for ROP.Conclusion: The heterogeneity between the regions regarding the incidence of ROP was reduced with increasing severity of ROP, and there was no heterogeneity regarding frequency of treatment for ROP, which is the most important issue for the children. We cannot exclude observer bias regarding mild ROP and ROP stage 3 in this study.
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6.
  • Edén, Ulla, et al. (författare)
  • Aniridia among children and teenagers in Sweden and Norway.
  • 2008
  • Ingår i: Acta Ophthalmologica. - : Wiley. - 1755-3768 .- 1755-375X. ; 86, s. 730-734
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose: To investigate patients under the age of 20 with aniridia in Sweden and Norway in order to estimate the prevalence of aniridia, to describe clinical signs and identify complications in the young, which will help improve diagnostic tools and treatment. Methods: A thorough search for patients with aniridia (of all ages) was performed. Sixty-two of the 181 patients were under the age of 20. Fifty-two of them were examined and they constituted the study population. Patient history was obtained and all participants underwent clinical ophthalmologic examination, including photography. Blood samples were taken for mutation analysis. Results: Epidemiological data are only based on the results in Sweden. The age-specific prevalence in Sweden was 1:47 000, male/female ratio was 0.57, mean age 12 years and median age 14 years. The proportion of sporadic cases including WAGR (Wilms tumour, Aniridia, Genitourinary abnormalities, Mental Retardation) and Gillespie syndrome (aniridia, cerebellar ataxia and mental retardation) was 48%. In the entire study population (Sweden and Norway), the mean visual acuity (VA) was 0.2 (range 0.04-0.9). We found VA < 0.3 in 80% and <0.1 in 18% of the patients. Twenty-two patients (42%) had one or more of the sight threatening complications such as cataract/lens luxation, corneal clouding or glaucoma. Conclusion: Descriptions of aniridia in the younger are rare. This study shows that aniridia seems to be more common than previously estimated and that some complications appear early in life. Watchfulness as regards these complications and regular examinations are essential even in the youngest.
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7.
  • Edén, Ulla, et al. (författare)
  • Corneal Involvement in Congenital Aniridia.
  • 2010
  • Ingår i: Cornea. - 1536-4798. ; Jul 1, s. 1096-1102
  • Tidskriftsartikel (refereegranskat)abstract
    • PURPOSE:: The purpose of this research is 2-fold. First of all, the level of keratopathy development in patients with congenital aniridia is studied. In addition, a correlation between the effects of ocular surgery on the severity of keratopathy is made. METHODS:: A thorough search for the total number of patients with aniridia in Sweden and Norway was performed. One hundred eighty-one patients were identified and 124 (69%) of these were examined. Three artificial eyes, 16 eyes with corneal transplants, and 1 eye with a corneal limbal allograft were excluded from the study. All participating patients underwent clinical ophthalmologic examinations, including photographs, and their medical history was recorded. A slit lamp was used to examine the presence of keratopathy. RESULTS:: Visible keratopathy was found in 80% of the eyes. Keratopathy that caused impaired visual acuity was found in 26% of the eyes. The study displayed a significant correlation between the level of keratopathy and the patient's age. A significant correlation between the level of keratopathy and intraocular surgery exists. This is irrespective of the patient's age. The study also found that irrespective of the patient's age, a significant correlation between the level of keratopathy and impaired corneal sensitivity exists. CONCLUSIONS:: This research identified the presence of visible keratopathy in 80% of eyes. In addition, 26% of eyes had a keratopathy level that caused visual disturbances. The study showed that the prevalence and severity of keratopathy increased with the patient's age. Further conclusions are that intraocular surgery increases the severity of keratopathy and that the severity of keratopathy is correlated to reduced corneal sensitivity. Finally, extreme care should be taken when selecting patients for intraocular surgery because this procedure can trigger the development of keratopathy.
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8.
  • Edén, Ulla, et al. (författare)
  • Epidemiology of aniridia in Sweden and Norway.
  • 2008
  • Ingår i: Acta Ophthalmologica. - : Wiley. - 1755-3768 .- 1755-375X. ; 86, s. 727-729
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose: To investigate the epidemiology of aniridia in the populations of Sweden and Norway. Methods: A thorough search for aniridia patients has been performed in Sweden and Norway. All participants had a clinical ophthalmological examination documented through photography. Blood samples were taken for mutation analysis and pedigrees were established. Results: A total of 181 patients with aniridia were identified in the two countries. This gives an age-specific prevalence of 1:72 000 in the entire region, 1:70 000 in Sweden and 1:76 000 in Norway. A total of 124 individuals (69%) were examined. Male/female ratio was 0.94 (Sweden 0.85 and Norway 1.2). Mean age of the examined patients was 29 years and median age 25 years. We did not find any significant age difference between the two countries. The mean visual acuity (VA) was 0.19 (Sweden 0.19 and Norway 0.18).The number of families with more than one affected member was 31 and the number of sporadic cases was 40. Conclusion: We have done a thorough search of the literature, but we have found no earlier studies describing aniridia in an entire country and only a few reports from larger areas. We assume that most aniridia patients have been found and the aniridia prevalence of 1:72 000 can be regarded as well supported. Further studies on other aspects of aniridia are in progress, and information from these can contribute to guidelines for the care of patients with this rare but serious disease.
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9.
  • Grönqvist, Sofia, et al. (författare)
  • Association between visual impairment and functional and morphological cerebral abnormalities in full-term children
  • 2001
  • Ingår i: Acta Ophthalmologica Scandinavica. - : Wiley. - 1395-3907. ; 79:2, s. 140-146
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose: To characterise the nature and degree of ocular disorders and cerebral morphological and functional abnormalities in a population-based group of visually impaired full-term pre-school children. Methods: Forty-five children who were born at full-term between 1989 and 1995 in Varmland, Sweden, were reported as being visually impaired. An ophthalmological examination was performed and clinical data regarding mental development and neurological disease were obtained for all children. Cerebral imaging was performed in 35 children, Results: Twenty-six per cent of the children were found to have ocular disorders only. Forty-two per cent had cerebral morphological abnormalities, verified by cerebral imaging, and 65% had signs of cerebral functional abnormalities. In total, 74% were found to have cerebral morphological and/or cerebral functional abnormalities. Conclusion: The majority of children with visual impairment, including children with ocular disorders, were found to have cerebral morphological and/or cerebral functional abnormalities. We suggest that any child with visual impairment should therefore undergo cerebral imaging and be examined by a paediatrician in order to establish the correct diagnosis.
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10.
  • Gyllén, Jenny, 1968, et al. (författare)
  • PECARE: Parental Feedback to Improve Congenital Cataract Care in Sweden
  • 2023
  • Ingår i: Journal of Pediatric Ophthalmology & Strabismus. - : SLACK, Inc.. - 0191-3913 .- 1938-2405. ; 60:4, s. 288-294
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose: To analyze non-directed parental feedback to health care providers responsible for pediatric cataract care in Sweden. Methods: A directed content analysis was used to analyze data consisting of text representing free comments provided by 40 parents. A deductive approach was employed by applying the model of balancing the child's inability and ability, which includes the categories mastering, collaborating, facilitating, and adapting. Results: Parents lacked piloting and self-management support. They experienced an absence of partnership with the health care team and not being taken seriously. They also felt abandoned by health care, resulting in emotional distress. Parents highlighted the impact of their social network and the challenges involved in accepting and adapting to the changes in everyday life. Conclusions: This study emphasizes the consequences of the lack of a caring partnership with health care professionals. Because parents act as mediators of care to the child with congenital cataract, persistence on the part of parents and a family-centered approach are essential for the child's visual development.
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11.
  • Haargaard, Birgitte, et al. (författare)
  • The Pediatric Cataract Register (PECARE): analysis of age at detection of congenital cataract
  • 2015
  • Ingår i: Acta Ophthalmologica. - : Wiley. - 1755-3768 .- 1755-375X. ; 93:1, s. 24-26
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose: To analyse and discuss screening for the detection of congenital cataract in two Nordic countries, Denmark and Sweden. Methods: Until 2011, in Denmark, no guideline concerning screening for congenital cataract existed. Since 2011, Danish guidelines regarding eye examination include examination with a pencil light at age 5 weeks, whereas newborn red reflex examination using a handheld ophthalmoscope is routine protocol in Swedish maternity wards. Data regarding age of referral were derived from the Pediatric Cataract Register (PECARE). All children operated on before 1 year of age between January 2008 and December 2012 were included. Statistical comparison of the different screening strategies was made. Results: The number of children undergoing surgery for congenital cataract before 1 year of age was 31 (17 bilateral cases) in Denmark and 92 (38 bilateral cases) in Sweden. The proportion was 14 per 100.000 children in Denmark and 16 in Sweden (p < 0.05). There was a statistically significant difference between Denmark and Sweden in the percentage of children referred within 42 days of birth (p < 0.0001) and within 100 days (p < 0.001). Conclusion: Due to the screening procedure with red reflex examination, congenital cataract in Swedish children is detected significantly earlier than in Danish children.
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12.
  • Heijl, Anders, et al. (författare)
  • Nordic research in ophthalmology.
  • 2005
  • Ingår i: Acta ophthalmologica Scandinavica. - : Wiley. - 1395-3907 .- 1600-0420. ; 83:3, s. 278-88
  • Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
    • Nordic ophthalmologists and vision scientists are active in many fields of eye research. This is most evident at the biannual Nordic Congress of Ophthalmology, most recently held in Malmö in June 2004. The authors here review some of the research in vision and ophthalmology presented at this meeting or published recently by Nordic scientists. This paper does not represent a comprehensive review of all Nordic research in the field, but attempts to give an overview of some of the activities underway in eye research in this part of the world.
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13.
  • Hellström, Ann, 1959, et al. (författare)
  • Extreme prematurity, treated retinopathy, bronchopulmonary dysplasia and cerebral palsy are significant risk factors for ophthalmological abnormalities at 6.5years of age
  • 2018
  • Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 107:5, s. 811-821
  • Tidskriftsartikel (refereegranskat)abstract
    • Aim: This study evaluated the contributions of various prenatal and postnatal predictive factors to a documented high prevalence of ophthalmological abnormalities in children aged 6.5 years who were born extremely preterm. Methods: We carried out a prospective population-based study of all children born in Sweden at a gestational age of 22+0 to 26+6weeks based on the Extremely Preterm Infants in Sweden Study. The main outcome measures were a combined score of visual impairment, refractive errors and strabismus at 6.5years of age. Models of univariate and multivariable regression were used to analyse potential prenatal and postnatal predictive factors at different clinically relevant time-points from one minute after birth to 30months. Results: We focused on 399 known extremely preterm survivors and compared them to 300 full-term controls. Significant antecedents for ophthalmological abnormalities included prematurity per se, retinopathy of prematurity that required treatment, severe bronchopulmonary dysplasia and cerebral palsy. Severe intraventricular haemorrhage was no longer a significant risk factor when we adjusted it for the 30-month cognitive and neuromotor development outcomes. Conclusion: This time-course risk analysis model showed a changing panorama of significant risk factors for ophthalmological abnormalities in children aged 6.5years who were born extremely preterm.
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14.
  • Holmström, Gerd E, et al. (författare)
  • Ophthalmologic Outcome at 30 Months Corrected Age of a Prospective Swedish Cohort of Children Born Before 27 Weeks of Gestation The Extremely Preterm Infants in Sweden Study
  • 2014
  • Ingår i: JAMA OPHTHALMOLOGY. - : American Medical Association (AMA). - 2168-6165 .- 2168-6173. ; 132:2, s. 182-189
  • Tidskriftsartikel (refereegranskat)abstract
    • IMPORTANCE Follow-up at 30 months corrected age reveals eye and visual problems in one-third of children born extremely prematurely (less than27 weeks gestation). OBJECTIVE To investigate the ophthalmologic outcome of extremely preterm children at 30 months corrected age. DESIGN, SETTING, AND PARTICIPANTS A prospective, population-based follow-up study (Extremely Preterm Infants in Sweden Study [EXPRESS]) was conducted in Sweden. The population included extremely preterm infants (less than27 weeks gestation) born in Sweden between 2004 and 2007, of whom 491 survived until age 2.5 years. Screening for retinopathy of prematurity (ROP) was performed in the neonatal period. At 30 months corrected age, an ophthalmologic assessment was performed in 411 of 491 children (83.7%). MAIN OUTCOMES AND MEASURES Visual acuity, manifest strabismus, and refractive errors were evaluated. RESULTS Visual impairment was identified in 3.1% of the children, and 1.0% were blind. Refractive errors, defined as myopia less than -3 diopters (D), hypermetropia greater than +3 D, astigmatism 2 D or more, and/or anisometropia 2 D or more, were found in 25.6% of the children, and 14.1% had manifest strabismus. There were significant associations between visual impairment and treated ROP (P = .02), cognitive disability (P less than .001), and birth weight (P = .02). Multiple regression analyses revealed significant associations between strabismus and treated ROP (P less than .001), cognitive disability (P less than .01), and cerebral palsy (P = .02). Refractive errors were significantly correlated with severity of ROP (right eye, P less than .001; left eye, P less than .01). Children who had been treated for ROP had the highest frequency (69.0%) of eye and visual abnormalities. CONCLUSIONS AND RELEVANCE One-third of the extremely prematurely born children in this study had some kind of eye or visual problems, such as visual impairment, strabismus, or major refractive error. Despite being born extremely preterm, the present cohort has a similar prevalence of blindness and visual impairment as in previous Swedish cohorts of children born less prematurely.
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15.
  • Holmström, Gerd E., et al. (författare)
  • Swedish National Register for Retinopathy of Prematurity (SWEDROP) and the Evaluation of Screening in Sweden
  • 2012
  • Ingår i: Archives of Ophthalmology. - Chicago, USA : American Medical Association (AMA). - 0003-9950. ; 130:11, s. 1418-1424
  • Tidskriftsartikel (refereegranskat)abstract
    • Objectives: To evaluate screening for retinopathy of prematurity (ROP) in Sweden and to investigate possible modifications of the present screening guidelines. Methods: Infants in Sweden with a gestational age (GA) of 31 weeks + 6 days or less are screened for ROP. Data from the Swedish national register for ROP (SWEDROP) during 2008 and 2009 were extracted and compared with a national perinatal quality register. Results: In SWEDROP, there were 1791 infants born before a GA of 32weeks from January 1, 2008, through December 31, 2009. Another 70 infants were registered in the perinatal quality register but not in SWEDROP (drop-out rate, 3.8% [70 of 1861 infants]). Seven infants died before termination of screening. In the final study cohort (1784 infants), 15.6% had mild ROP and 8.5% had severe ROP. Treatment was performed in 4.4% of the infants, none of whom had a GA at birth of more than 28 weeks. Nine infants with a GA of more than 28 weeks at birth developed stage 3 ROP, which regressed spontaneously. The total number of examinations was 9286 (964 in infants with a GA of 31 weeks), and the mean (range) number of examinations of each infant was 5.2 (1-30). Conclusions: The SWEDROP, a quality register for ROP, has a national coverage (ie, participation) of 96%. Data from 2008 to 2009 show that it seems possible to reduce the upper limit for screening in Sweden by 1 week, including only infants with a GA of 30 weeks + 6 days or less. However, such a change should be combined with a strong recommendation to neonatologists to refer also severely ill and more "mature" infants.
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16.
  • Holmström, Gerd, et al. (författare)
  • Evaluation of new guidelines for ROP screening in Sweden using SWEDROP - a national quality register
  • 2015
  • Ingår i: Acta Ophthalmologica. - : Wiley. - 1755-375X .- 1755-3768. ; 93:3, s. 265-268
  • Tidskriftsartikel (refereegranskat)abstract
    • PurposeTo investigate whether recent Swedish guidelines for Retinopathy of Prematurity (ROP) screening, that is, a gestational age (GA) at birth of <31weeks (w), are applicable in a new national cohort of prematurely born infants. MethodsSWEDROP is a national register for ROP, initiated in 2006. The present paper reports on data from the register on various aspects of screening for ROP in infants born between 2010 and 2011 and compares the results with those for a previously published cohort born between 2008 and 2009. ResultsDuring the study period, 1744 infants were screened for ROP. Mean GA was 28.4w (22-31), and mean birth weight was 1239g (382-2615). Screening started at postnatal age (PNA) 5.4w (0.4-13.3) and postmenstrual age (PMA) 33.8 w (24.9-50.1) Mean number of examinations was 5.4 per infant (1-38). Mild (stages 1-2) and severe ( stage 3) ROP was found in 15.4% and 8.7%, respectively. Treatment was performed in 4.2% (73/1744) of the infants, but in none with a GA of 30weeks or more. The first treatment was performed at a mean PNA and PMA of 12.7 w (7.7-25.4) and 37.4 w (32.1-51.4), respectively. ConclusionsRecently introduced new guidelines for ROP screening in Sweden remain applicable. Reassuringly, in infants born between 2010 and 2011, incidence of ROP, frequency and timing of treatment, frequency and timing of examinations and national coverage of ROP screening remained almost identical to those for a previous cohort from 2008 to 2009. The two SWEDROP cohorts provide a basis for discussion among Swedish ophthalmologists and neonatologists on the question of further lowering the upper screening limit with 1week.
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17.
  • Holmström, Gerd, 1951-, et al. (författare)
  • Increased frequency of retinopathy of prematurity over the last decade and significant regional differences
  • 2018
  • Ingår i: Acta Ophthalmologica. - : Wiley. - 1755-375X .- 1755-3768. ; 96:2, s. 142-148
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose: Retinopathy of prematurity (ROP) causes childhood blindness globally in prematurely born infants. Although increased levels of oxygen supply lead to increased survival and reduced frequency of cerebral palsy, increased incidence of ROP is reported. Methods: With the help of a Swedish register for ROP, SWEDROP, national and regional incidences of ROP and frequencies of treatment were evaluated from 2008 to 2015 (n = 5734), as well as before and after targets of provided oxygen changed from 85-89% to 91-95% in 2014. Results: Retinopathy of prematurity (ROP) was found in 31.9% (1829/5734) of all infants with a gestational age (GA) of <31 weeks at birth and 5.7% of the infants (329/5734) had been treated for ROP. Analyses of the national data revealed an increased incidence of ROP during the 8-year study period (p = 0.003), but there was no significant increase in the frequency of treatment. There were significant differences between the seven health regions of Sweden, regarding both incidence of ROP and frequency of treatment (p < 0.001). Comparison of regional data before and after the new oxygen targets revealed a significant increase in treated ROP in one region [OR: 2.24 (CI: 1.11-4.49), p = 0.024] and a borderline increase in one other [OR: 3.08 (CI: 0.99-9.60), p = 0.052]. Conclusion: The Swedish national ROP register revealed an increased incidence of ROP during an 8-year period and significant regional differences regarding the incidence of ROP and frequency of treatment.
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18.
  • Holmström, Gerd, 1951-, et al. (författare)
  • New modifications of Swedish ROP guidelines based on 10-year data from the SWEDROP register
  • 2020
  • Ingår i: British Journal of Ophthalmology. - : BMJ. - 0007-1161 .- 1468-2079. ; 104:7, s. 943-949
  • Tidskriftsartikel (refereegranskat)abstract
    • BACKGROUND/AIMS:During the last decade, improved neonatal care has resulted in increased survival of the most immature infants and improved health of more mature infants. We hypothesise that this has affected incidence and treatment of retinopathy of prematurity (ROP), enabling guidelines for screening to be modified.METHODS: In Sweden, all infants with gestational age (GA) at birth ≤30 weeks are screened for ROP. Results are registered in a web-based register, Swedish National ROP Register, with a coverage rate of 97%. Incidence of ROP and frequency of treatment, aspects on natural course of ROP and number of examinations, are calculated in relation to GA at birth in infants born during 2008-2017.RESULTS: Of 7249 infants, 31.9% (2310) had ROP and 6.1% (440) were treated. No infant with GA 30 weeks was treated. Incidence of ROP remained similar, but frequency of treatment increased (p=0.023). Over time, GA and birth weight were reduced in infants with ROP and with treated ROP. In the most immature infants, postmenstrual age was lower and postnatal age was higher when any ROP and stage 3 ROP were first detected (p<0.001). At treatment, postmenstrual but not postnatal age of the infant was associated with GA (p<0.001). During the 10-year period, 46 038 examinations were performed.CONCLUSION: Modification of Swedish guidelines is proposed, including only infants with a GA of <30 weeks and postponing the first examination with 1 week in infants with GA 26-29 weeks. This would spare many infants from stressful examinations and reduce eye examinations with at least 20%.
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19.
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20.
  • Källén, Karin, et al. (författare)
  • Ophthalmologic Outcome of Extremely Preterm Infants at 6.5 Years of Age Extremely Preterm Infants in Sweden Study (EXPRESS)
  • 2016
  • Ingår i: Jama Ophthalmology. - : American Medical Association (AMA). - 2168-6165 .- 2168-6173. ; 134:5, s. 555-562
  • Tidskriftsartikel (refereegranskat)abstract
    • IMPORTANCE This follow-up study of extremely preterm (EPT) children (<27 weeks' gestational age [GA] at birth) revealed major eye and visual problems in 37.9%(147 of 388) of all EPT infants and in 55.4%(67 of 121) of the most immature subgroups at 6.5 years of age. These major eye and visual problems were strongly associated with treatment-requiring retinopathy of prematurity (ROP). OBJECTIVES To investigate the ophthalmologic outcome of a national cohort of EPT children at 6.5 years of age and to evaluate the impact of prematurity and ROP. DESIGN, SETTING, AND PARTICIPANTS All surviving EPT children born in Sweden between April 1, 2004, and March 31, 2007, were included and compared with a matched term control group, as part of a prospective national follow-up study. MAIN OUTCOMES AND MEASURES Visual acuity, refraction in cycloplegia, and manifest strabismus were evaluated and compared with GA at birth and with treatment-requiring ROP. RESULTS The study cohort comprised 486 participants. The mean (SD) GA of the children who were included was 25 (1) weeks, and 45.7%(222 of 486) were female. At a median age of 6.6 years, 89.3%(434 of 486) of eligible EPT children were assessed and compared with 300 control group children. In the EPT group, 2.1%(9 of 434) were blind, 4.8%(21 of 434) were visually impaired according to the World Health Organization criteria, and 8.8% (38 of 434) were visually impaired according to the study criteria. Strabismus was found in 17.4% (68 of 390) and refractive errors in 29.7%(115 of 387) of the EPT children compared with 0% (0 of 299) and 5.9% (17 of 289), respectively, of the control children (P<.001). Altogether at 6.5 years of age, 37.9%(147 of 388) of the EPT children had some ophthalmologic abnormality compared with 6.2%(18 of 290) of the matched control group (95% CI of the difference, 26.1%-37.2%). When treatment-requiring ROP was adjusted for, no significant association between GA and visual impairment could be detected. For refractive errors, the association with GA remained after adjustment for treatment-requiring ROP (odds ratio, 0.72; 95% CI, 0.58-0.91 for each 1-week increment). CONCLUSIONS AND RELEVANCE In a Swedish national cohort of EPT children at 6.5 years of age, major eye and visual problems were frequently found. Treatment-requiring ROP was a stronger impact factor than GA on visual impairment and strabismus, but not on refractive errors, as a whole. In modern neonatal intensive care settings, ophthalmologic problems continue to account for a high proportion of long-term sequelae of prematurity.
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21.
  • Larsson, Eva, 1961-, et al. (författare)
  • Ophthalmological outcome of 6.5 years children treated for retinopathy of prematurity: a Swedish register study
  • 2024
  • Ingår i: British Journal of Ophthalmology. - : BMJ. - 0007-1161 .- 1468-2079. ; 108:1, s. 137-142
  • Tidskriftsartikel (refereegranskat)abstract
    • AimsTo determine the ophthalmological outcome at 6.5 years of age in children treated for retinopathy of prematurity (ROP), and registered in the national Swedish National Register for ROP register. MethodsData on ROP, treatment and ophthalmological outcome were retrieved from the register. Visual acuity (VA), refractive errors and strabismus, together with visual impairment (VI) and any significant eye problem, defined as VA >0.5 logarithm of the minimal angle of resolution (logMAR) and/or strabismus and/or any refractive error were analysed. Risk factors such as sex, gestational age (GA), birth weight SD score, number of treatments and retreatments, postnatal age and postmenstrual age at first treatment were analysed. ResultsFollow-up data were available in 232 of 270 children born between 2007 and 2014 who had been treated for ROP. VI (VA >0.5 logMAR) was found in 32 (14%), strabismus in 82 (38%), refractive errors in 114 (52%) and significant eye problem in 143 (65%) children. Retreatment was a risk factor for VI and refractive errors. Male sex and neonatal brain lesion were risk factors for strabismus. An additional week of GA at birth reduced the risk for refractive errors, strabismus and significant eye problems. ConclusionThe results of the present study revealed a high number of eye problems in children treated for ROP, emphasising the need for long-term follow-up. Retreatment of ROP was a risk factor for VI, and emphasises the importance of an accurate first treatment for the long-term ophthalmological outcome.
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22.
  • Larsson, Eva, 1961- (författare)
  • Short- and Long-Term Follow-Up of Ophthalmological Findings in Preterm Infants and Children
  • 2004
  • Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
    • In a prospective population-based study in Stockholm County, 1998-2000, the incidence of retinopathy of prematurity (ROP) was investigated and was found to be 36% in prematurely-born infants with a birth weight of ≤ 1500 grams. Compared to a study performed ten years ago, the overall incidence was unchanged, but was reduced in “mature” infants and increased in immature ones. The incidence of ROP was 25% in infants with a gestational age of ≤ 32 weeks at birth. The main risk factors for ROP were the gestational age at birth, followed by the birth weight. Current guidelines for ROP screening in Sweden were modified.A 10-year follow-up study of the ophthalmological findings in prematurely-born children, previously included in a prospective population-based incidence study of ROP, was performed. The children were compared with full-term ones. Prematurely-born children ran a four times higher risk of refractive errors than full-term ones. The cryotreated children had the highest risk, but those without ROP also had more refractive errors than the full-terms. Within the group of prematurely-born children, the cryotreated ones had the highest prevalence of myopia, astigmatism and anisometropia, but no difference was found regarding hypermetropia.The visual acuity of prematurely-born children was poorer than that of the full-terms. The cryotreated children and those with neurological complications had the most marked reduction, but the children without ROP and neurological findings also had a poorer visual outcome than the full-terms. The prevalence of visual impairment was 1.8% among the prematurely-born children, and was due to ROP in half the cases and cerebral lesions in the others. The cryotreated children had constricted peripheral visual fields compared to the untreated prematurely-born and full-term children. The central visual fields tended to be reduced in the prematurely-born children compared to the full-terms, but no difference was observed within the preterm group.
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23.
  • Lundgren, Pia, et al. (författare)
  • High rate and large intercentre variability in retreatment of retinopathy of prematurity in infants born < 24 gestational weeks
  • 2021
  • Ingår i: BMJ Open Ophthalmology. - : BMJ Publishing Group Ltd. - 2397-3269. ; 6:1
  • Tidskriftsartikel (refereegranskat)abstract
    • Objective Prematurity is a major risk factor for retinopathy of prematurity (ROP). We aimed to elucidate ROP prevalence, treatment and retreatment in infants born before 24 gestational age (GA) weeks in a Swedish cohort. Methods and analysis Infants with completed ROP screening, born at <24 GA weeks, 2007-2018 in Sweden were included. Data of GA, birth weight (BW), sex, neonatal morbidities, maximal ROP stage, aggressive posterior ROP (APROP), ROP treatments, treatment modality and treatment centre were retrieved. Results In total, 399 infants, with a mean GA of 23.2 weeks (range 21.9-23.9) and a mean BW of 567 g (range 340-874), were included. ROP was detected in 365 (91.5%) infants, 173 (43.4%) were treated for ROP and 68 of 173 (39.3%) were treated more than once. As the first treatment, 142 (82.0%) received laser and 29 (16.1%) received intravitreal injection of antivascular endothelial growth factor (anti-VEGF). Retreatment was performed after first laser in 46 of 142 (32.4%) and in 20 of 29 (69.0%) after first anti-VEGF treatment. Retreatment rate was not associated with GA, BW or sex but with APROP, treatment method (anti-VEGF) and treatment centre where the laser was performed (p<0.001). Twenty eyes progressed to retinal detachment, and two infants developed unilateral endophthalmitis after anti-VEGF treatment. Conclusion Infants, born at <24 weeks GA, had high rates of treatment-warranting ROP and retreatments. Treatment centre highly influenced the retreatment rate after laser indicating that laser treatment could be improved in some settings.
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24.
  • Magnusson, Gunilla, et al. (författare)
  • Congenital cataract screening in maternity wards is effective: evaluation of the Paediatric Cataract Register of Sweden
  • 2013
  • Ingår i: Acta Pædiatrica. - : Wiley. - 1651-2227 .- 0803-5253. ; 102:3, s. 263-267
  • Tidskriftsartikel (refereegranskat)abstract
    • Aim To study which eye-screening protocol prevails in Swedish maternity/neonatal wards, evaluate efficacy in a prospective study and compare results with earlier Swedish retrospective results. Methods Surveys were sent in 2006 to maternity/neonatal and women's health departments regarding screening policy. Response frequency was 96% (122/127). Data were derived from the Paediatric Cataract Register (PECARE), Sweden. All Swedish children diagnosed with congenital cataract and operated on before 1year of age between January 2007 and December 2009 were included. Statistical comparison with earlier retrospective results was performed. Results Eye screening is a routine protocol at a rate of 90% of Swedish maternity wards. Sixty-one children were included in the study. An increase was shown in case referrals from maternity wards compared to 10years ago (64% vs. 50%). Detection was performed within 6weeks of age in 75% of the cases. A significant difference between the probabilities of early referral (0.38; p<0.001, < 6weeks of age) and early surgery (0.36; p<0.001) (PECARE) was found in comparison with the historical data of no maternity-ward screening. Well-baby clinics were instrumental in early detection, as well. Conclusion Eye screening in maternity wards is effective. Clear Swedish directives are to be preferred.
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25.
  • Magnusson, Gunilla, 1968, et al. (författare)
  • Evaluation of screening procedures for congenital cataracts.
  • 2003
  • Ingår i: Acta paediatrica (Oslo, Norway : 1992). - : Wiley. - 0803-5253 .- 1651-2227. ; 92:12, s. 1468-73
  • Tidskriftsartikel (refereegranskat)abstract
    • AIM: To evaluate the efficacy of two different Swedish screening procedures for early detection of congenital cataracts in comparison with no screening. METHODS: Children born between January 1992 and December 1998 in Swedish regions with an established eye-screening routine procedure, diagnosed with congenital cataract, and operated on before 1 y of age, were included in a retrospective study. Age at referral and age at time of the operation were compared between regions using different screening procedures: screening in the maternity wards (Region 1), at the well-baby clinics (Region 2) and one region without any screening (Region 3). RESULTS: Seventy-two children were included in the study. Concerning early diagnosis and surgery, Region 1 differed significantly from Regions 2 and 3, which were more similar and were combined for further analysis. The difference in detected cases was greatest at 21 d of age (55% vs 18%; p < 0.001), but persisted even at 100 d of age (78% vs 64%; p < 0.02). Region 1 screening resulted in more and earlier cases detected than the other two regions (22 vs 15 per 100,000 births). In 72% of all cases, surgery was performed in response to referrals from either the maternity wards (36%), or the well-baby clinics (36%). However, half of the cases from the well-baby clinics were detected too late, i.e. at > 100 d. CONCLUSION: Eye screening in the maternity ward is preferable to well-baby clinic screening and to no screening at all, since it leads to early detection. Screening should also be performed routinely at well-baby clinics within the period when successful treatment is possible.
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26.
  • Magnusson, Gunilla, 1968, et al. (författare)
  • The Paediatric Cataract Register (PECARE): an overview of operated childhood cataract in Sweden and Denmark
  • 2018
  • Ingår i: Acta Ophthalmologica. - : Wiley. - 1755-375X .- 1755-3768. ; 96:1, s. 51-55
  • Tidskriftsartikel (refereegranskat)abstract
    • Aim: To report basic epidemiological data concerning surgically treated childhood cataract in Sweden and Denmark. Methods: Data were derived from the Paediatric Cataract Register (PECARE), a binational, web-based surgical register representing Sweden and Denmark. All children operated before 8years of age between 1 January 2007 and 31 December 2013 were included. Age-specific prevalence per 100000 population was calculated. Results: A total 574 operations in 213 boys (51.7%) and 199 girls (48.3%), altogether 412 children, were registered, the vast majority (n=395/412; 95.9%) being individuals with congenital/infantile cataract. Of these 412, a total of 294 (147 boys and 147 girls) were Swedish and 118 (66 boys and 52 girls) were Danish. The age-specific prevalence of operated cataract in Sweden was 31/100 000 and in Denmark 28/100 000. In 454 of 574 eyes (79.1%), the cataract was dense. Altogether, 266 of 574 (46.3%) were operated during the first year of life, 193 during the first 12weeks representing 33.6% of all operations. A primary intraocular lens (IOL) implantation was done in altogether 411 of 574 eyes (71,6%). In total, 210 unilateral cataract operations (210/574; 36.6%) were performed. Persistent fetal vasculature (PFV) was present in 64 of 193 (33.1%) of those with a congenital unilateral cataract. In 84 individuals (84/395; 21.3%) with congenital or infantile cataract, a coexisting disorder was found. Conclusion: The age-specific binational prevalence of operated congenital/infantile cataract in Sweden and Denmark is 30/100000. About half of the operations are performed within the first year of life, one-third within the first 3months. In our study population, a primary IOL was implanted in the majority of cases. © 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd
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27.
  • Magnusson, Gunilla, 1968, et al. (författare)
  • The prevalence of visual axis opacification in the Swedish Pediatric Cataract Register
  • 2024
  • Ingår i: ACTA OPHTHALMOLOGICA. - 1755-375X .- 1755-3768.
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpos o report on the occurrence of postoperative visual axis opacification (VAO) in children younger than 5 years of age operated for cataract in Sweden, and to analyse correlations with age at surgery and surgical method.Methods Data were derived from the Swedish Pediatric Cataract Register (PECARE). All children operated on between 1 January 2007 and 31 December 2020 were included. Follow-ups at 1, 2 and 5 years of age were analysed.Results Cataract surgery were performed on 770 eyes belonging to 549 children (n = 282 boys, 51.4%); 327/770 (42.5%) of the children underwent surgery before 3 months of age and 216/770 (28%) before 6 weeks of age. Data on 881 follow-up visits were registered. At the follow up-visits at 1, 2 and 5 years of age, VAO was present in 154/349 (44.1%), 41/323 (12.7%) and 25/208 (12%). The majority of the children with VAO underwent cataract surgery before age 6 months, with a predominance before age 2 months. Primary IOL was implanted in 601/770 (78%) of eyes; 40.8% had an acrylic one-piece lens, 31.8% had a bag-in-the-lens IOL, 21.9% were aphakic and 5.2% had an acrylic three-piece lens. Implantation of a bag-in-the-lens IOL was related to a significantly lower occurrence of VAO compared to other types of IOL, including aphakia (p < 0.0002).Conclusion Our results are in accordance with the literature. Primary bag-in-the-lens IOL implantation before 2 years of age seems adequate and safe, with a low occurrence of VAO, and can thus be continued as routine in Sweden.
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28.
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29.
  • Norman, Mikael, et al. (författare)
  • Prevalence of Severe Visual Disability Among Preterm Children With Retinopathy of Prematurity and Association With Adherence to Best Practice Guidelines
  • 2019
  • Ingår i: JAMA Network Open. - : American Medical Association (AMA). - 2574-3805. ; 2:1, s. 186801-186801
  • Tidskriftsartikel (refereegranskat)abstract
    • Importance: Retinopathy of prematurity (ROP) can cause severe visual disability even in high-resource settings. A better understanding of the prevalence and processes leading to ROP-induced severe visual impairment may help health care professionals design preventive measures.Objectives: To determine the prevalence of severe visual disability among children born preterm in Sweden, evaluate adherence to best practice, and determine the health system's structural capacity.Design, Setting, and Participants: Population-based, nationwide cohort study of 1 310 227 children born between January 1, 2004, and December 31, 2015, in Sweden, of whom 17 588 (1.3%) were born very preterm (<32 weeks of gestation). Children born preterm with a verified diagnosis of severe visual disability had their medical records reviewed for evaluation of ROP screening, diagnosis, and treatment. In addition, a questionnaire on structural capacity was sent to all ophthalmology departments.Exposures: Stages 4 and 5 ROP.Main Outcomes and Measures: The primary outcome was prevalence of severe visual disability (visual acuity ≤20/200 for both eyes) associated with ROP stages 4 and 5. Secondary outcomes included adherence to national ROP guidelines using a predefined protocol with 15 key performance indicators for screening, diagnosis, and treatment; assessment of whether visual disability was deemed avoidable; and examination of structural capacity, including information on equipment and facilities, staffing, and patients.Results: Seventeen children (10 boys; mean [range] birth weight, 756 [454-1900] g; mean [range] gestational age, 25 [22-33] weeks) became severely visually disabled because of ROP, corresponding to a prevalence of 1 in 1000 very preterm infants (<32 weeks of gestational age) and 1 in 77 000 for all live births. Severe visual impairment was considered potentially avoidable in 11 of 17 affected children (65%) owing to untimely or no screening, missed diagnosis, or untimely and suboptimal treatment. Large variations in infrastructure (facilities, guidelines, staffing, and annual patient numbers) were also identified as potential contributors to these findings.Conclusions and Relevance: Retinopathy of prematurity still causes severe visual disability in Sweden, resulting in 1 affected infant per 1000 very preterm births. In most of these infants, noncompliance with best practice was identified, indicating that a significant proportion could have been avoided.
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30.
  • Nyström, Alf, et al. (författare)
  • The Swedish National Pediatric Cataract Register (PECARE): incidence and onset of postoperative glaucoma
  • 2020
  • Ingår i: Acta Ophthalmologica. - : Wiley. - 1755-375X .- 1755-3768. ; 98:7, s. 654-661
  • Tidskriftsartikel (refereegranskat)abstract
    • Purpose The aim was to report cumulative incidence and time of onset of postoperative glaucoma in a paediatric early cataract surgery cohort. Methods Data were retrieved from the Pediatric Cataract Register (PECARE), a prospective register of Swedish cataract operations before 8 years of age. All eyes with surgery between January 2007 and December 2014 and a registered follow-up were included. Cataracts caused by uveitis, trauma or coexisting congenital glaucoma were excluded. Glaucoma was defined as early onset if diagnosed within a year after surgery and late onset if diagnosed later. Results The study included 288 eyes in 207 children (106 girls), 81 with bilateral and 126 with unilateral cataracts, with a mean follow-up of 3.31 +/- 1.77 years. Of the 288, 168 (58.3%) had surgery before 3 months of age; most of these 92.3% (155/168) were defined as dense, 208 (72.2%) were below 1 year of age. Cumulative incidence of surgically treated glaucoma among individuals was 23.7% (49/207). Median time to glaucoma onset was 0.91 years (range: 0.05-4.97 years) for eyes. Early-onset glaucoma was found in 98 % (63/64), and late onset in 2% (1/64). Conclusion In this paediatric cataract cohort, a majority of eyes had surgery before 3 months of age (58.3%). Secondary glaucoma-onset peaked within the first postoperative year, with a cumulative incidence of 23.7%. Surgery performed after the first month of life, resulted in a lower glaucoma rate. Long-term follow-up will reveal whether the low rate of late-onset glaucoma with early surgery will last, and if so, the consequences.
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31.
  • Pivodic, Aldina, 1978, et al. (författare)
  • Development and validation of a new clinical decision support tool to optimize screening for retinopathy of prematurity
  • 2022
  • Ingår i: British Journal of Ophthalmology. - : BMJ Publishing Group Ltd. - 0007-1161 .- 1468-2079. ; 106:11, s. 1573-1580
  • Tidskriftsartikel (refereegranskat)abstract
    • BACKGROUND/AIMS: Prematurely born infants undergo costly, stressful eye examinations to uncover the small fraction with retinopathy of prematurity (ROP) that needs treatment to prevent blindness. The aim was to develop a prediction tool (DIGIROP-Screen) with 100% sensitivity and high specificity to safely reduce screening of those infants not needing treatment. DIGIROP-Screen was compared with four other ROP models based on longitudinal weights.METHODS: Data, including infants born at 24-30 weeks of gestational age (GA), for DIGIROP-Screen development (DevGroup, N=6991) originate from the Swedish National Registry for ROP. Three international cohorts comprised the external validation groups (ValGroups, N=1241). Multivariable logistic regressions, over postnatal ages (PNAs) 6-14 weeks, were validated. Predictors were birth characteristics, status and age at first diagnosed ROP and essential interactions.RESULTS: ROP treatment was required in 287 (4.1%)/6991 infants in DevGroup and 49 (3.9%)/1241 in ValGroups. To allow 100% sensitivity in DevGroup, specificity at birth was 53.1% and cumulatively 60.5% at PNA 8 weeks. Applying the same cut-offs in ValGroups, specificities were similar (46.3% and 53.5%). One infant with severe malformations in ValGroups was incorrectly classified as not needing screening. For all other infants, at PNA 6-14 weeks, sensitivity was 100%. In other published models, sensitivity ranged from 88.5% to 100% and specificity ranged from 9.6% to 45.2%.CONCLUSIONS: DIGIROP-Screen, a clinical decision support tool using readily available birth and ROP screening data for infants born GA 24-30 weeks, in the European and North American populations tested can safely identify infants not needing ROP screening. DIGIROP-Screen had equal or higher sensitivity and specificity compared with other models. DIGIROP-Screen should be tested in any new cohort for validation and if not validated it can be modified using the same statistical approaches applied to a specific clinical setting.
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32.
  • Pivodic, Aldina, et al. (författare)
  • Prognostic Value of Parenteral Nutrition Duration on Risk of Retinopathy of Prematurity
  • 2023
  • Ingår i: JAMA ophthalmology. - : American Medical Association (AMA). - 2168-6165 .- 2168-6173. ; 141:8, s. 716-716
  • Tidskriftsartikel (refereegranskat)abstract
    • Importance  The prognostic impact of parenteral nutrition duration (PND) on retinopathy of prematurity (ROP) is not well studied. Safe prediction models can help optimize ROP screening by effectively discriminating high-risk from low-risk infants.Objective  To evaluate the prognostic value of PND on ROP; to update and validate the Digital ROP (DIGIROP) 2.0 birth into prescreen and screen prediction models to include all ROP-screened infants regardless of gestational age (GA) and incorporate PND; and to compare the DIGIROP model with the Weight, IGF-1, Neonatal, and ROP (WINROP) and Postnatal Growth and ROP (G-ROP) models.Design, Setting, and Participants  This retrospective study included 11 139 prematurely born infants from 2007 to 2020 from the Swedish National Registry for ROP. Extended Poisson and logistic models were applied. Data were analyzed from August 2022 to February 2023.Main Outcomes and Measures  Any ROP and ROP requiring treatment were studied in relation to PND. ROP treatment was the outcome in DIGIROP models. Sensitivity, specificity, area under the receiver operating characteristic curve, and adjusted OR (aOR) with 95% CI were the main measures. Internal and external validations were performed.Results  Of 11 139 screened infants, 5071 (45.5%) were girls, and the mean (SD) gestational age was 28.5 (2.4) weeks. ROP developed in 3179 infants (29%), treatment was given in 599 (5%), 7228 (65%) had PND less than 14 days, 2308 (21%) had PND for 14 days or more, and 1603 (14%) had unknown PND. PND was significantly correlated with ROP severity (Spearman r = 0.45; P < .001). Infants with 14 days or more of PND vs less than 14 days had faster progression from any ROP to ROP treatment (adjusted mean difference, −0.9 weeks; 95% CI, −1.5 to −0.3; P = .004). Infants with PND for 14 days or more vs less than 14 days had higher odds of any ROP (aOR, 1.84; 95% CI, 1.62-2.10; P < .001) and of severe ROP requiring treatment (aOR, 2.20; 95% CI, 1.73-2.80; P < .001). Among all 11 139 infants, the DIGIROP 2.0 models had 100% sensitivity (95% CI, 99.4-100). The specificity was 46.6% (95% CI, 45.6-47.5) for the prescreen model and 76.9% (95% CI, 76.1-77.7) for the screen model. G-ROP as well as the DIGIROP 2.0 prescreen and screen models showed 100% sensitivity on a validation subset (G-ROP: sensitivity, 100%; 95% CI, 93-100; DIGIROP prescreen: sensitivity, 100%; 95% CI, 93-100; DIGIROP screen: sensitivity, 100%; 95% CI, 93-100), whereas WINROP showed 89% sensitivity (95% CI, 77-96). Specificity for each prediction model was 29% (95% CI, 22-36) for G-ROP, 38% (95% CI, 32-46) for DIGIROP prescreen, 53% (95% CI, 46-60) for DIGIROP screen at 10 weeks, and 46% (95% CI, 39-53) for WINROP.Conclusion and Relevance  Based on more than 11 000 ROP-screened infants born in Sweden, PND of 14 days or more corresponded to a significantly higher risk of having any ROP and receiving ROP treatment. These findings provide evidence to support consideration of using the updated DIGIROP 2.0 models instead of the WINROP or G-ROP models in the management of ROP.
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33.
  • Pivodic, Aldina, 1978, et al. (författare)
  • Prognostic Value of Parenteral Nutrition Duration on Risk of Retinopathy of Prematurity Development and Validation of the Revised DIGIROP Clinical Decision Support Tool
  • 2023
  • Ingår i: JAMA ophthalmology. - : AMER MEDICAL ASSOC. - 2168-6165 .- 2168-6173. ; 141:8, s. 716-724
  • Tidskriftsartikel (refereegranskat)abstract
    • IMPORTANCE The prognostic impact of parenteral nutrition duration (PND) on retinopathy of prematurity (ROP) is not well studied. Safe prediction models can help optimize ROP screening by effectively discriminating high-risk from low-risk infants. OBJECTIVE To evaluate the prognostic value of PND on ROP; to update and validate the Digital ROP (DIGIROP) 2.0 birth into prescreen and screen prediction models to include all ROP-screened infants regardless of gestational age (GA) and incorporate PND; and to compare the DIGIROP model with the Weight, IGF-1, Neonatal, and ROP (WINROP) and Postnatal Growth and ROP (G-ROP) models. DESIGN, SETTING, AND PARTICIPANTS This retrospective study included 11 139 prematurely born infants from 2007 to 2020 from the Swedish National Registry for ROP. Extended Poisson and logistic models were applied. Data were analyzed from August 2022 to February 2023. MAIN OUTCOMES AND MEASURES Any ROP and ROP requiring treatment were studied in relation to PND. ROP treatment was the outcome in DIGIROP models. Sensitivity, specificity, area under the receiver operating characteristic curve, and adjusted OR (aOR) with 95% CI were the main measures. Internal and external validations were performed. RESULTS Of 11 139 screened infants, 5071 (45.5%) were girls, and the mean (SD) gestational age was 28.5 (2.4) weeks. ROP developed in 3179 infants (29%), treatment was given in 599 (5%), 7228 (65%) had PND less than 14 days, 2308 (21%) had PND for 14 days or more, and 1603 (14%) had unknown PND. PND was significantly correlated with ROP severity (Spearman r = 0.45; P < .001). Infants with 14 days or more of PND vs less than 14 days had faster progression from any ROP to ROP treatment (adjusted mean difference, -0.9 weeks; 95% CI, -1.5 to -0.3; P = .004). Infants with PND for 14 days or more vs less than 14 days had higher odds of any ROP (aOR, 1.84; 95% CI, 1.62-2.10; P < .001) and of severe ROP requiring treatment (aOR, 2.20; 95% CI, 1.73-2.80; P < .001). Among all 11 139 infants, the DIGIROP 2.0 models had 100% sensitivity (95% CI, 99.4-100). The specificity was 46.6%(95% CI, 45.6-47.5) for the prescreen model and 76.9%(95% CI, 76.1-77.7) for the screen model. G-ROP as well as the DIGIROP 2.0 prescreen and screen models showed 100% sensitivity on a validation subset (G-ROP: sensitivity, 100%; 95% CI, 93-100; DIGIROP prescreen: sensitivity, 100%; 95% CI, 93-100; DIGIROP screen: sensitivity, 100%; 95% CI, 93-100), whereas WINROP showed 89% sensitivity (95% CI, 77-96). Specificity for each prediction model was 29% (95% CI, 22-36) for G-ROP, 38%(95% CI, 32-46) for DIGIROP prescreen, 53%(95% CI, 46-60) for DIGIROP screen at 10 weeks, and 46%(95% CI, 39-53) for WINROP. CONCLUSION AND RELEVANCE Based on more than 11 000 ROP-screened infants born in Sweden, PND of 14 days or more corresponded to a significantly higher risk of having any ROP and receiving ROP treatment. These findings provide evidence to support consideration of using the updated DIGIROP 2.0 models instead of the WINROP or G-ROP models in the management of ROP.
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34.
  • Pivodic, Aldina, 1978, et al. (författare)
  • Validation of DIGIROP models and decision support tool for prediction of treatment for retinopathy of prematurity on a contemporary Swedish cohort
  • 2023
  • Ingår i: British Journal of Ophthalmology. - : BMJ. - 0007-1161 .- 1468-2079. ; 107:8, s. 1132-1138
  • Tidskriftsartikel (refereegranskat)abstract
    • Background/Aims Retinopathy of prematurity (ROP) is currently diagnosed through repeated eye examinations to find the low percentage of infants that fulfil treatment criteria to reduce vision loss. A prediction model for severe ROP requiring treatment that might sensitively and specifically identify infants that develop severe ROP, DIGIROP-Birth, was developed using birth characteristics. DIGIROP-Screen additionally incorporates first signs of ROP in different models over time. The aim was to validate DIGIROP-Birth, DIGIROP-Screen and their decision support tool on a contemporary Swedish cohort. Methods Data were retrieved from the Swedish national registry for ROP (2018-2019) and two Swedish regions (2020), including 1082 infants born at gestational age (GA) 24 to <31 weeks. The predictors were GA at birth, sex, standardised birth weight and age at the first sign of ROP. The outcome was ROP treatment. Sensitivity, specificity and area under the receiver operating characteristic curve (AUC) with 95% CI were described. Results For DIGIROP-Birth, the AUC was 0.93 (95% CI 0.90 to 0.95); for DIGIROP-Screen, it ranged between 0.93 and 0.97. The specificity was 49.9% (95% CI 46.7 to 53.0) and the sensitivity was 96.5% (95% CI 87.9 to 99.6) for the tool applied at birth. For DIGIROP-Screen, the cumulative specificity ranged between 50.0% and 78.7%. One infant with Beckwith-Wiedemann syndrome who fulfilled criteria for ROP treatment and had no missed/incomplete examinations was incorrectly flagged as not needing screening. Conclusions DIGIROP-Birth and DIGIROP-Screen showed high predictive ability in a contemporary Swedish cohort. At birth, 50% of the infants born at 24 to <31 weeks of gestation were predicted to have low risk of severe ROP and could potentially be released from ROP screening examinations. All routinely screened treated infants, excluding those screened for clinical indications of severe illness, were correctly flagged as needing ROP screening.
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35.
  • Riise, R, et al. (författare)
  • The phenotype in Norwegian patients with Bardet-Biedl syndrome with mutations in the BBS4 gene
  • 2002
  • Ingår i: Archives of Ophthalmology. - 0003-9950. ; 120:10, s. 1364-1367
  • Tidskriftsartikel (refereegranskat)abstract
    • Objective: To describe the phenotype of the Bardet-Biedl syndrome in patients with mutations in the BBS4 gene. Methods: We examined 3 pairs of siblings with Bardet-Biedl syndrome in whom 3 different mutations in the BBS4 gene were detected, 2 of which were homozygous for the mutation. Results: All patients had an increased body mass index. The obesity varied between families from moderate to severe. All of the males had hypogenitalism. All had brachydactyly and similar dental anomalies. Polydactyly was present in 5 of the 6 patients. The number and location of the extra digits varied even between siblings. The intelligence varied between families and was within the normal range in 4 individuals. One male had spinal stenosis with paraparesis of his legs. Four patients had increased blood pressure, but only I had impaired renal function. Severe retinitis pigmentosa with onset in early childhood was present in all patients. There were few abnormal retinal pigmentary deposits even at advanced stages. Conclusions: The phenotype of patients with BBS4 mutations consists of severe retinitis pigmentosa, variable obesity, brachydactyly with variable polydactyly, small or missing teeth, genital hypoplasia, and cardiovascular disease. The combinations of clinical signs are mostly independent of the individual BBS4 mutation and can vary even within pairs of siblings. It is possible that there is a characteristic appearance of the ocular fundus in patients with BBS4 mutations.
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36.
  • Ståhl Hallengren, Christina, et al. (författare)
  • An extra cysteine in one of the non calcium binding EGF-like motifs of the FBN1 polypeptide is connected to a novel variant of Marfan syndrome
  • 1994
  • Ingår i: Journal of Clinical Investigation. - 0021-9738. ; 94, s. 709-713
  • Tidskriftsartikel (refereegranskat)abstract
    • We present here a family with a clinical phenotype resembling Marfan syndrome (MFS), and displaying joint contracture and episodes of knee joint effusions, but lacking the cardiovascular features of the syndrome. The phenotype of this family represents a unique mixture of connective tissue symptoms, some of which are found in classical MFS and some of which are typical of dominant ectopia lentis. Linkage analyses suggested a linkage (LOD score 2.4; theta = 0) between the phenotype of the family and a polymorphic marker in the vicinity of the fibrillin locus on chromosome 15 (FBN1). Furthermore, a novel transition mutation was identified in the FBN1 gene in all the affected members of the family. In contrast to the majority of fibrillin mutations reported so far, this mutation substitutes a cysteine for arginine, producing an extra cysteine in one of the non-calcium-binding EGF-like motifs of the fibrillin polypeptide, most probably disturbing the formation of one of the three disulfide bridges known to be essential for the normal conformation of this motif.
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37.
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38.
  • Tornqvist, Kristina, et al. (författare)
  • Ocular Malformations or Poor Visual Acuity in Children Born After In Vitro Fertilization in Sweden
  • 2010
  • Ingår i: American Journal of Ophthalmology. - : Elsevier Science B.V., Amsterdam.. - 0002-9394 .- 1879-1891. ; 150:1, s. 23-26
  • Tidskriftsartikel (refereegranskat)abstract
    • PURPOSE: To follow up children born after in vitro fertilization (IVF) with respect to eye malformations and poor visual acuity. DESIGN: Observational cohort study based on Swedish health registers. METHODS: Congenital eye malformations were studied in 32 091 children born from 1982 through 2007 and severe visual impairment was studied in 24 628 children born from 1985 through 2005 after IVF in Sweden. Comparisons were made with all children born in Sweden during corresponding periods with adjustment for various confounders. The main outcome measure was the presence of a congenital eye malformation and poor visual acuity. RESULTS: Thirty-six (1.1 per 1000) IVF infants with ocular malformations were identified, and the risk, compared with non-IVF children, was not increased when adjusted for maternal age, parity, smoking, and body mass index (odds ratio, 1.05; 95% confidence interval, 0.75 to 1.47). Severe visual impairment was identified in 25 cases (1.0 per 1000), and the risk increase was statistically significant (odds ratio, 1.65; 95% confidence interval, 1.12 to 2.45) and was only slightly reduced when adjustment as above was made (odds ratio, 1.55; 95% confidence interval, 1.04 to 2.32). When adjustment was made for known length of unwanted childlessness, the OR decreased to 1.15 (95% confidence interval, 0.61 to 2.16). Only 3 of the 25 children with visual impairment had ocular malformations. CONCLUSIONS: Although there is an increased risk for visual impairment among children born after IVF, the individual risk is small and may be secondary to parental characteristics. No increased risk for eye malformations was found. (Am J Ophthalmol 2010;150:23-26.
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39.
  • Tornqvist, Kristina, et al. (författare)
  • Optic nerve hypoplasia: Risk factors and epidemiology.
  • 2002
  • Ingår i: Acta Ophthalmologica Scandinavica. - : Wiley. - 1395-3907. ; 80:3, s. 300-304
  • Tidskriftsartikel (refereegranskat)abstract
    • OBJECTIVES: To study the epidemiology of optic nerve hypoplasia. DESIGN AND METHODS: Children with optic nerve hypoplasia and visual impairment were identified through the Swedish Register of Visually Impaired Children. Pre- and perinatal characteristics were obtained from the Medical Birth Registry and by scrutinizing pregnancy and delivery records. Clinical characteristics of children with optic nerve hypoplasia are described. The following risk factors were studied: maternal age, parity, maternal smoking, gestational duration, birth weight, delivery method, Apgar score, maternal disease during pregnancy, drugs used in early pregnancy. RESULTS: Young maternal age, first parity, maternal smoking, preterm birth and factors associated with preterm birth were risk factors for optic nerve hypoplasia. There was an indicated association with the use of fertility drugs and antidepressant drugs. CONCLUSIONS: Optic nerve hypoplasia is apparently associated not only with other anomolies, notably of the central nervous system, but also with signs of general disturbance in fetal development.
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40.
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41.
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42.
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43.
  • Wackerberg, D., et al. (författare)
  • Analysis of age at detection and outcomes of dense unilateral congenital cataract surgery for children on the paediatric cataract register
  • 2023
  • Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 112:2, s. 277-285
  • Tidskriftsartikel (refereegranskat)abstract
    • AimAnalysis of age at time of detection and surgery of dense unilateral cataract and investigation of best-corrected visual acuity (BCVA) in a nationwide register-based cohort study, based on the routine of maternity ward eye screening. MethodsData were derived from the Paediatric Cataract Register (PECARE). All children (n = 54) diagnosed with dense congenital unilateral cataract between January 2007 and September 2014 who had surgery before 1 year of age, and for whom 5-year follow-up records were available, were included. ResultsThe majority, 35/54 (65%), were detected and operated on before age 6 weeks and 30/35 (86%) were referred from maternity wards. Visual acuity (VA) >= 0.5 (decimal, 0.3 logMAR) was found in 7/53 (13%) of the cohort at age 5 years; further, 19 children achieved VA >= 0.1 (decimal, 1.0 logMAR) (36%) and 19 children VA < 0.05 (decimal, 1.30 logMAR) (36%). Ten-year follow-up records were available for 17/53 (32%) children; 1/17 (6%) achieved VA >= 0.5 (decimal, 0.3 logMAR), 4/17 (24%) VA >= 0.3-<0.5 (decimal, 0.52-0.30 logMAR), 3/17 (18%) VA >= 0.05-0.1 (decimal, 1.30-1.0 logMAR) and 10/17 (59%) VA < 0.05 (decimal, 1.30 logMAR). ConclusionA total of 90% of the children were detected with cataract within 100 days of birth and 80% were operated on within this period. This study showed better visual acuity in those treated for dense unilateral cataracts than previously reported in an earlier Swedish cohort study.
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