Adherence to oxygen saturation targets increased in preterm infants when a higher target range and tighter alarm limits were introduced.

AIM: European consensus guidelines published in May 2013 recommended a target peripheral capillary oxygen saturation (SpO2 ) range of 90-95% for preterm infants. These were incorporated into guidelines at the Karolinska University Hospital, Sweden, in November 2013. This study compared clinical practice before and after those local guidelines. METHODS: We included infants who were born between 23 + 0 and 30 + 6 weeks from January 1, 2013 to December, 31 2015 and received intensive care in two Karolinska units. The lower saturation target of 88-92% and alarm limits of 85-95% used before November 2013 were compared to the new higher saturation target of 90-95% and alarm limits of 89-96%. RESULTS: Data from 399 infants were analysed. The mean SpO2 was 92.4% with the higher target (n = 301) and 91.1% with the lower target (n = 98). Using the higher instead of lower target meant that the SpO2 was within the prescribed target range more frequently (51% versus 30%) and the proportion of time with SpO2 >95% was increased by 9% (95% confidence interval 7-11%, p < 0.001). CONCLUSION: The higher saturation target and tighter alarm limits led to higher mean oxygen saturation, increased adherence to the target and increased time with hyperoxaemia.

Ophthalmological outcome and visuospatial ability in very preterm children measured at 2.5 years corrected age.

AIM: To investigate the ophthalmological outcome of very preterm children at 2.5 years corrected age (CA) and perform a test of visuospatial and cognitive abilities. METHODS: A population-based, prospective study (LOVIS study) in Uppsala County, Sweden, comprised 111 very preterm children (<32 w gestational age [GA]) born between 1 January 2004 and 31 December 2007. Ophthalmic evaluations were undertaken in 98/109 children (89.9%) alive at 2.5 years. Spatial cognition was investigated with a test of five alternative blocks in 48 preterm and 25 term-born children. RESULTS: Visual impairment, strabismus or refractive errors, were found in 12% of the children. None of the children were blind in both eyes. Logistic regression analyses revealed significant associations between strabismus and periventricular leucomalacia/intraventricular haemorrhage (OR 9.6, p = 0.025) and between refractive errors and severe retinopathy of prematurity (OR 9.8, p = 0.011) and GA (OR 0.763, p = 0.034). Oval and rectangular blocks were significantly more difficult to insert into a box for preterm than full-term children (p = 0.048 and 0.013, respectively). There was a significant correlation between total scores for the five blocks and GA at birth (p = 0.035). CONCLUSION: Eye and visual problems were found in 12% of the preterm children at 30 months CA. Preterm children had difficulties with blocks of complex shapes.

Depression and age as predictors of patient-reported outcome in a multidisciplinary rehabilitation programme for chronic musculoskeletal pain.

BACKGROUND: The recommended treatment for chronic musculoskeletal pain is multidisciplinary, with a cognitive approach. The aim of this study was to investigate health-related quality of life (HRQoL) outcome after a multidisciplinary treatment with a cognitive approach. METHODS: A total of 131 subjects who participated in a multidisciplinary rehabilitation programme (2005-2008) were studied at baseline and after six months, using the Short Form Short Form 36-item Health Survey questionnaire (SF-36) as primary outcome (HRQoL), and the Hospital Anxiety and Depression Scale (HAD) and pain as secondary outcomes and possible baseline predictors for HRQoL. RESULTS: Complete data were available for 97 subjects (85 women, mean age [SD] 44.6 [9.7] years). The SF-36 subscales physical function (PF), general health (GH), vitality (VT), social function (SF) and mental health (MH), the visual analogue scale for pain and the HAD improved significantly (p < 0.05) at follow-up compared with baseline. A pre-treatment probable depression (HAD score ≥11) was associated with a favourable outcome of the SF-36 subscales PF (odds ratio [OR] 5.6; p = 0.01), VT (OR 4.3; p = 0.02) and MH (OR 3.6; p = 0.02). A probable anxiety (HAD score ≥11) was associated with a favourable outcome of PF (OR 2.6; p = 0.05). There was an even stronger association for younger subjects (20-45 years), with probable depression scores at baseline and a favourable HRQoL outcome at follow up. CONCLUSION: This multidisciplinary rehabilitation programme, using a non-pharmacological cognitive approach, seemed to yield a better outcome concerning HRQoL measures in younger subjects with higher depression scores at baseline. This information is important for clinics when tailoring a multidisciplinary rehabilitation programme for patients with musculoskeletal chronic pain.

Noonan syndrome and neurofibromatosis type I in a family with a novel mutation in NF1.

Noonan syndrome (NS) and neurofibromatosis type I (NF1) belong to a group of clinically related disorders that share a common pathogenesis, dysregulation of the RAS-MAPK pathway. NS is characterized by short stature, heart defect, pectus deformity and facial dysmorphism, whereas skin manifestations, skeletal defects, Lisch nodules and neurofibromas are characteristic of NF1. Both disorders display considerable clinical variability. Features of NS have been observed in individuals with NF1 -a condition known as neurofibromatosis-Noonan syndrome (NFNS). The major gene causing NFNS is NF1. Rarely, a mutation in PTPN11 in addition to an NF1 mutation is present. We present the clinical and molecular characterization of a family displaying features of both NS and NF1, with complete absence of neurofibromas. To investigate the etiology of the phenotype, mutational analysis of NF1 was conducted, revealing a novel missense mutation in exon 24, p.L1390F, affecting the GAP-domain. Additional RAS-MAPK pathway genes were examined, but no additional mutations were identified. We confirm that NF1 mutations are involved in the etiology of NFNS. Furthermore, based on our results and previous studies we suggest that evaluation of the GAP-domain of NF1 should be prioritized in NFNS.

Contrast sensitivity in 10 year old preterm and full term children: a population based study.

AIMS: To determine the contrast sensitivity (CS) in 10 year old prematurely born children, previously included in a population based study on the incidence of retinopathy of prematurity (ROP), and in full term controls. METHODS: This study included 205 prematurely born children and 215 children born at term, from the same geographical area and study period. CS was assessed monocularly with the Vistech 6500 test at five spatial frequencies (1.5-18 cycles/deg). RESULTS: Prematurely born children had statistically significant lower CS at all frequencies, compared to full term ones. The mean differences in logarithmic CS were 0.03 (1.5 cycles/deg), 0.09 (3 cycles/deg), 0.10 (6 cycles/deg), 0.12 (12 cycles/deg), and 0.19 (18 cycles/deg). Even when the children with ROP and neurological disorders were excluded there was a difference between the two groups. Children who had been treated with cryotherapy had the lowest CS. CONCLUSION: CS was lower in 10 year old prematurely born children than in full term ones of the same age. Whether this finding affects their visual function in daily life is uncertain.

Screening for retinopathy of prematurity: evaluation and modification of guidelines.

AIMS: To evaluate current screening guidelines for ROP (retinopathy of prematurity) and to determine whether they can be modified. METHODS: In accordance with the authors' present criterion, infants born in Stockholm County, Sweden, from 1 August 1998 to 31 July 2000, with a gestational age of < or =32 weeks, were screened for ROP. The effectiveness of screening was studied. RESULTS: The incidence of ROP was 25.5% in this study. A dropout group comprising almost 20% of the population studied (< or =32 weeks), was never referred, were lost to follow up, or died before screening was completed. No infant with a gestational age of >31 weeks at birth developed severe ROP (stages 3-5) and no infant with a gestational age of >29 weeks was treated for ROP. CONCLUSION: 80% of infants in this population with a gestational age at birth of < or =32 weeks, the current screening criterion, were effectively screened for ROP. The authors recommend that the screening criterion be lowered to

Incidence of ROP in two consecutive Swedish population based studies.

AIMS: To prospectively analyse the present incidence of ROP (retinopathy of prematurity) in a well defined geographical area in Sweden, and to compare it with that from a decade earlier in exactly the same area. METHODS: Infants born between 1 August 1998 and 31 July 2000 with a birth weight of 1500 g or less were studied. They were screened for ROP from 5 weeks of postnatal age until the retina was entirely vascularised. The incidence of ROP, with its various stages, was compared with that of a previous (1988-90) population based study in the same geographical area. RESULTS: The incidence of ROP in the present study was 36.4% (mild (18.2%) and severe ROP (18.2%)), which was similar to that of the previous study. Gestational age at birth remained the most important risk factor for ROP. We found a change in the distribution of ROP. The probability of ROP, particularly severe ROP, was highest in the most immature infants while it was lower in the least immature ones. CONCLUSIONS: The incidence of ROP remained the same in two consecutive population based studies. The more "mature" infants developed ROP, particularly severe ROP, less often, while the most immature infants had ROP more often, particularly severe ROP.

Actinomyces suimastitidis sp. nov., isolated from pig mastitis.

An unusual Actinomyces-like bacterium originating from a pig with mastitis was subjected to a polyphasic taxonomic investigation. The morphological and biochemical characteristics of the organism were consistent with its preliminary assignment to the genus Actinomyces but it did not appear to correspond to any recognized species. PAGE analysis of whole-cell proteins confirmed the phenotypic distinctiveness of the bacterium and 16S rRNA gene sequence analysis demonstrated that it represents a hitherto unknown sub-line amongst a cluster of Actinomyces species which embraces Actinomyces canis, Actinomyces georgiae, Actinomyces hyovaginalis, Actinomyces meyeri, Actinomyces odontolyticus, Actinomyces radingae and Actinomyces turicensis. Based on phylogenetic and phenotypic evidence, it is proposed that the unknown bacterium isolated from pig mastitis be classified as Actinomyces suimastitidis sp. nov. The type strain of Actinomyces suimastitidis is CCUG 39279T (= CIP 106779T).

Ocular manifestations of incontinentia pigmenti.

PURPOSE: The study aimed to evaluate the ocular manifestations in patients with incontinentia pigmenti (IP). METHODS: Thirty patients from different parts of Sweden participated. Orthoptic and ocular examinations were performed as well as evaluation of refraction and visual acuity. RESULTS: Ocular manifestations, probably associated with IP, were found in 77% (23/30) of the patients. Thirteen had serious or vision-threatening eye manifestations in one eye, of whom 7 were totally blind in that eye from retinal detachments. Ten patients had minor retinal and/or corneal changes. CONCLUSION: Ocular lesions in patients with IP may be serious and lead to blindness because of retinal disease. Ophthalmological follow-up is essential in the neonatal period and such a programme is recommended.

Ophthalmological follow up of preterm infants: a population based, prospective study of visual acuity and strabismus.

BACKGROUND/AIMS: Prematurely born infants are known to have an increased rate of ophthalmological morbidity. The aim of the present study was to investigate visual acuity and ocular alignment in a population of preterm infants in a geographical area, in infants with and without retinopathy of prematurity (ROP). METHODS: A prospective population based study of ophthalmological status of preterm infants with a birth weight of 1500 g or less was performed during 3.5 years, with examinations at 6, 18, 30, and 42 months of corrected age. Visual acuity was tested using linear optotypes. Multiple regression analyses were used to analyse independent risk factors for poor vision and strabismus. RESULTS: Poor vision (< 0.3) was detected in 2.5% (6/237) of the children. Of these, only two (0.8%) had a severe visual impairment (< 0.1). Strabismus occurred in 13.5% (31/229). Children with cryotreated ROP and neurological complications ran the highest risk of poor vision and strabismus, according to multiple regression analysis. Among children without a history of ROP or neurological complications, 34% had a visual acuity < 0.7 and 5.9% had strabismus, compared with 61% and 22%, respectively, among the children with ROP or neurological complications. CONCLUSIONS: The overall incidence of subnormal vision and strabismus in children born prematurely was higher than in a full term population of the same age. On the basis of this study, follow up of all preterm infants screened for ROP is recommended and general guidelines are suggested.

Capillary haemangiomas in association with morning glory disc anomaly.

PURPOSE AND METHOD: Capillary haemangiomas in children are usually isolated, but may have systemic associations. This accords with our finding of ocular malformations, especially "morning glory disc anomaly" (MGDA), in three children which are described. RESULTS: All three children had extensive capillary haemangiomas in combination with MGDA in one eye. One of the children also had microphthalmos in one eye and a partial agenesis of the corpus callosum. Another child had sclerocornea in one eye and congenital heart defects. CONCLUSION: An association of extensive capillary haemangiomas with MGDA is described. The embryological timing of such defects as well as investigation of the children is discussed. Extensive haemangiomas have not previously, to our knowledge, been described in association with MGDA.

Neonatal risk factors for retinopathy of prematurity--a population-based study.

PURPOSE: The aim of the study was to evaluate possible neonatal risk factors for retinopathy of prematurity (ROP) in a population-based group of preterm, very low birth weight, infants. METHOD: The main study group included 202 single-born infants with a birth weight of 1500 grams or less. A group of 57 twins were also described. Selected risk factors were extracted from the neonatal records. RESULTS: Univariate analysis revealed an association between ROP and respiratory distress syndrome (RDS), bronchopulmonary dysplasia (BPD), septicaemia, intraventricular bleeding, and the use of ventilator as well as continuous positive airway pressure. In a stepwise logistic regression analysis, however, only gestational age at birth, birth weight and BPD were significantly associated with ROP. CONCLUSION: Prematurity per se remains the strongest risk factor for ROP.

Maternal risk factors for retinopathy of prematurity--a population-based study.

BACKGROUND: The aim of the study was to investigate maternal risk factors for retinopathy of prematurity (ROP) and also to describe risk factors for prematurity per se in our population. DESIGN: A retrospective, population-based study. MATERIAL AND METHODS: The study group included the mothers (identified with the help of a National Birth Register) of 202 single-born preterm infants with a birth weight of 1500 grams or less, from a previous population-based study. From the same register we also received information on 307 mothers of full term pregnancies for description of risk factors for preterm birth. Data from antenatal clinics, labor rooms and neonatal units were studied. RESULTS: Univariate analysis of maternal risk factors for ROP revealed a few positive findings. In a further logistic multiple regression analysis, apart from gestational age at birth and birth weight, only essential hypertension prior to pregnancy was a predicting risk factor (odds/ ratio 8.36, 95% confidence interval 1.62-43.0). CONCLUSIONS: Prematurity per se remains the strongest risk factor for ROP.

Patterns of visual impairment associated with lesions of the preterm infant brain.

The visual function of 42 children with haemorrhagic and/or ischaemic cerebral lesions acquired before a gestational age of 35 weeks was examined and related to cranial ultrasound in the neonatal period and to MRI and neurodevelopmental status at follow-up. All 37 children with abnormal ultrasound scans and one of the five with normal ultrasound scans showed impairment of one or more aspects of visual function. While impaired acuity was more frequent among infants with MRI evidence of visual pathway damage, this was not an invariable finding. Normal or near-normal visual acuity did not preclude the presence of other functional visual deficits. The authors conclude that preterm cerebral insults may produce a variety of visual difficulties, the pattern and severity of which cannot be predicted on imaging. Each child therefore requires individual assessment of multiple aspects of visual function.

A population based, prospective study of the development of ROP in prematurely born children in the Stockholm area of Sweden.

A prospective population based study including 260 children with a birth weight of 1500 g or less was performed in the Stockholm county. The total incidence of retinopathy of prematurity (ROP) was 40.4%, while severe ROP--that is, stage 3 or more, was seen in 20.0%. Cryotherapy was performed in 10.8%. Logistic regression analysis revealed independent association of both gestational age and birth weight with ROP. The association of gestational age and ROP was significantly stronger, indicating that the degree of immaturity of the eye is a main predictive factor for the development of ROP. The purpose of a general screening for ROP is to identify children requiring cryotherapy. Based on these results it seems appropriate to include children with a gestational age of 32 weeks or less in such a programme and a first examination at 5-6 weeks of postnatal age is suggested.

Regressed retinopathy of prematurity in children aged 5-10 years.

Regressed retinopathy of prematurity (ROP) and its sequelae has been analysed in pre-term born children with a birth weight < 1500 g and/or < 33 weeks of gestational age, born in Stockholm during 1976-81. At the age of 5-10 years, we found that 134 of 528 preterm born children (25.4%) had been at departments of ophthalmology in Stockholm for various reasons. These children were re-examined and a good eye fundus information could be obtained in 105. Moderate and severe regressed ROP was found in 48 and 13 children, respectively. In the Stockholm area the frequency of children with severe ROP (earlier called cicatricial ROP) was 2.5% out of the above risk group of 528. Two children were blind from ROP (0.4%). Ten children had a visual acuity < or = 0.3 in the worse eye (1.9%) and 7 of them had ROP changes. Children with a birth weight < 1000 g had the highest frequency of ROP (severe 11.6% and moderate 18.6%) and ocular complications. The results show that preterm born children often present ocular complications, and therefore they need a close follow-up.