| 1. |
- Winbo, Annika, 1978-, et al.
(författare)
-
Low incidence of sudden cardiac death in a Swedish Y111C type 1 long-QT syndrome population
- 2009
-
Ingår i: Circulation. - Philadelphia, PA : Lippincott Williams & Wilkins. - 1942-325X .- 1942-3268. ; 2:6, s. 558-564
-
Tidskriftsartikel (refereegranskat)abstract
- Background: A 10% cumulative incidence and a 0.3% per year incidence rate of sudden cardiac death in patients younger than 40 years and without therapy have been reported in type 1 long-QT syndrome. The Y111C-KCNQ1 mutation causes a severe phenotype in vitro, suggesting a high-risk mutation. This study investigated the phenotype among Y111C-KCNQ1 mutation carriers in the Swedish population with a focus on life-threatening cardiac events.Methods and Results: We identified 80 mutation carriers in 15 index families, segregating the Y111C-KCNQ1 mutation during a national inventory of mutations causing the long-QT syndrome. Twenty-four mutation carriers <40 years experienced syncope (30%). One mutation carrier had an aborted cardiac arrest (1.25%). No case of sudden cardiac death was reported during a mean nonmedicated follow-up of 25±20 years. This corresponds to a low incidence rate of life-threatening cardiac events (0.05%/year versus 0.3%/year, P=0.025). In 8 Y111C families connected by a common ancestor, the natural history of the mutation was assessed by investigating the survival over the age of 40 years for 107 nonmedicated ascertained mutation carriers (n=24) and family members (n=83) born between 1873 and 1968. In total, 4 deaths in individuals younger than 40 years were noted: 1 case of noncardiac death and 3 infant deaths between 1873 and 1915.Conclusions: The dominant-negative Y111C-KCNQ1 mutation, associated with a severe phenotype in vitro, presents with a low incidence of life-threatening cardiac events in a Swedish population. This finding of discrepancy emphasizes the importance of clinical observations in the risk stratification of long-QT syndrome.
|
|
| 2. |
- Birkeland, Anna-Lena, 1955-, et al.
(författare)
-
The complexity of the psychosocial situation in children and adolescents with heart disease
- 2005
-
Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 94:10, s. 1495-1501
-
Tidskriftsartikel (refereegranskat)abstract
- Aim: To describe the psychosocial situation of children/adolescents with heart disease and their families, an inventory method was worked out.Methods: Ninety-seven children/adolescents with congenital heart disease (CHD) were graded into three categories with respect to complexity of CHD. Group I included 42 patients with malformations requiring standardized operations. Group II included 20 patients with more complicated malformations, and group III included 35 patients with very complex malformations. The patients were compared with controls without heart disease, matched for age and gender. The psychosocial impact of CHD was measured by the inventory.Results: The most frequent problems in the patient group were healthcare and treatment-related needs (71/97) in the external sphere, family symptoms (68/97) in the interpersonal sphere, and somatic symptoms (19/97) in the personal sphere. Corresponding numbers in the controls were treatment-related needs (15/97), family (9/97) and somatic symptoms (25/97). Fifty per cent of the symptoms in the patient groups were mild, 30% moderate and 20% severe. The most severe symptoms were found in the interpersonal sphere, where family symptoms constituted the most severe variable. The frequency of severe problems in the personal sphere was 11% in the patients and 1% in the controls. This inventory method differentiates the grades of medical complexity both regarding number and severity of psychosocial symptoms. It indicates severe personal problems in the most complex group and shows that they have severe personal problems independent of family problems.Conclusion: This study elucidates the psychosocial complexity in children/adolescents with CHD, which has clinical implications in developing a psychosocial care programme.
|
|
| 3. |
|
|
| 4. |
|
|
| 5. |
- Ekvall, Tomas, et al.
(författare)
-
Avfallsprevention och giftfri miljö
- 2009
-
Rapport (övrigt vetenskapligt/konstnärligt)abstract
- Avfallsprevention genom ökad materialeffektivitet ger ofta miljöfördelar, t ex i form av minskade utsläpp av växthusgaser, eftersom det bidrar till att hålla nere energiintensiv materialproduktion. Syftet med detta projekt är att undersöka om ökad materialeffektivitet också bidrar till minskade utsläpp av farliga ämnen. Minskar eller ökar utsläppen av farliga ämnen om man ökar materialeffektiviteten? Finns det någon tydlig korrelation eller ej? Med farliga ämnen menar vi framförallt ämnen som hanteras av miljömålet Giftfri miljö och i Naturvårdsverkets strategi för giftfria och resurssnåla kretslopp (GRK), men också andra ämnen som påverkar människors hälsa. Vi utgår ifrån olika strategier för ökad materialeffektivitet (se Tabell S). För varje strategi identifierar vi exempel på hur den leder till ökade eller minskade utsläpp av farliga ämnen. Vi lyfter särskilt fram exempel och slutsatser som rör byggsektorn och livsmedelskedjan, eftersom de lyfts fram som prioriterade områden i GRK-strategin. Utifrån de identifierade exemplen drar vi slutsatsen att det finns många fall där ökad materialeffektivitet också bidrar till en mer giftfri miljö (se Tabell S). I vissa fall ger materialeffektiviteten viktiga miljöfördelar utöver att själva materialproduktionen minskar. Det gäller t ex fallet med småbilar. Det finns dock fall när ökad materialeffektivitet vare sig bidrar till giftfrihet eller andra miljöförbättringar. Det finns även fall där ökad materialeffektivitet bidrar till minskat energibehov, men ändå riskerar att öka användningen av farliga ämnen och/eller spridningen av dem i miljön. Slutligen finns risk för så kallade rebound-effekter om den ökade materialeffektiviteten är kostnadseffektiv. Vår samlade bedömning är att ökad materialeffektivitet ofta ger miljöfördelar också i form av minskade utsläpp av farliga ämnen. Sambandet verkar dock vara svagare än mellan materialeffektivitet och minskade utsläpp av växthusgaser. Osäkerheten är också större, eftersom frågan om farliga utsläpp är mer komplex. Man bör t ex ta hänsyn till farligheten hos ämnet och inte bara till den använda eller utsläppta mängden. Frågan är dessutom mindre utforskad. Ämnet för vår studie omfattar i princip alla material, alla produkter och alla produktionsprocesser. Vi har långt ifrån täckt ämnet fullständigt. Viktiga delar av studien är också relativt ytliga. Våra slutsatser skulle bli säkrare och mer välgrundade om studien breddas med fler exempel och/eller fördjupas på de punkter där den är ytlig. I förlängningen kan också systemanalyser vara motiverade, t ex inför implementeringen av EUs nya ramdirektiv om avfall (EU 2008) i svensk rätt.
|
|
| 6. |
- Grahn, K, et al.
(författare)
-
Attitudes about dental care among parents whose children suffer from severe congenital heart disease : a case-control study
- 2006
-
Ingår i: International Journal of Paediatric Dentistry. - 0960-7439 .- 1365-263X. ; 16:4, s. 231-238
-
Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVES: To examine attitudes and experiences of parents whose children have complex congenital heart disease (CHD) with respect to dental health information and advice, dental care, and service and to compare the results with data from an age- and gender-matched control group without any medical problems. SETTING: Faculty of Medicine (Paediatric Cardiology and Paediatric Dentistry), Umea University, Umea, Sweden. SAMPLE AND METHOD: Each group comprised parents of 33 children; the children's mean age was 9.4 years. All the cases and the controls resided in the county of Vasterbotten, northern Sweden. Data were collected with a questionnaire with 20 joint questions to both groups and four additional questions to the CHD group. RESULTS: Of the 20 joint questions, significant differences were displayed in the following areas: the professional group that provided the parents with dental health information and advice (P < 0.01), attitudes to reception at the dental clinic, and experience of sedation before operative dental treatment (P < 0.05). Parents to 11 children with CHD who were patients at a specialist clinic for paediatric dentistry scored the reception at the dental clinic as excellent in nine cases and satisfactory in two, compared to excellent (3), satisfactory (11), decent (4), and poor (4) among those who were patients in general dental practice (P < 0.01). No statistically significant differences in educational level or in parental experience of dental health were noted between the two groups (P > 0.05). CONCLUSION: Children with CHD in northern Sweden mainly receive their dental health information from a physician or a dentist, and healthy children mainly receive information from a dental hygienist indicating that children with CHD are given priority in the dental care system. Parental attitudes to reception in the dental service differed, and parents of healthy children scored the reception at the dental clinic better than parents of children with CHD. It is suggested that children with severe CHD should receive dental care in clinics for paediatric dentistry, particularly at early ages.
|
|
| 7. |
- Granberg, Marie, et al.
(författare)
-
Activities in daily living and schoolwork task performance in children with complex congenital heart disease.
- 2008
-
Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 97:9, s. 1270-4
-
Tidskriftsartikel (refereegranskat)abstract
- AIM: To examine if children with complex congenital heart disease (CHD) differ significantly from healthy children in performance of activities in daily living (ADL) and schoolwork tasks. METHODS: Fourteen children with complex CHD (3-14 years) were matched in pairs by age and gender to healthy children (n = 14). The ADL Taxonomy was used to evaluate ability to perform ADL and the School Version of the Assessment of Motor and Process Skills (AMPS) to evaluate quality of schoolwork task performance. RESULTS: The children with complex CHD had significantly lower mean ADL, school motor and school process performance. Eight of the pairs differed significantly in school motor ability, ten in school process performance and nine in ability to perform ADL. CONCLUSION: This study indicates that there is a difference in overall performance both in ADL and schoolwork task performance between children with complex CHD and healthy children. These findings may suggest that early interventions should be provided for children with complex CHD, who often do not receive such services, in order to enhance participation in childhood ADL and schoolwork.
|
|
| 8. |
|
|
| 9. |
- Rydberg, Annika, et al.
(författare)
-
Can analysis of heart rate variability predict arrhythmia in children with fontan circulation?
- 2008
-
Ingår i: Pediatric Cardiology. - : Springer. - 0172-0643 .- 1432-1971. ; 29:1, s. 50-55
-
Tidskriftsartikel (refereegranskat)abstract
- The aim of this study was to examine whether changes in heart rate variability (HRV) can predict arrhythmias in children who have undergone the Fontan procedure. The study included 15 children with total cavopulmonary connection. All examinations included echocardiography and 24-h ambulatory electrocardiogram with power spectral analysis of HRV and Poincaré plots (plots of each R-R interval as a function of the previous R-R interval). Six patients developed supraventricular tachycardia (four or more consecutive supraventricular beats). One patient was excluded from the study due to the development of bradycardia, necessitating placement of a pacemaker. Compared to the arrhythmia group, the patients without arrhythmias (n = 8) exhibited a significant difference in the standard deviation of instantaneous beat-to-beat R-R interval variability (p = 0.02). Poincaré plots of patients without arrhythmias showed a typical torpedo- or comet-shaped pattern, whereas the arrhythmia group showed a more complex pattern. Although this study examined only a few subjects, needing confirmation by larger studies, it does indicate that patients who develop arrhythmias after receiving the Fontan procedure show a different Poincaré pattern compared to the nonarrhythmic group—even before the arrhythmia can be detected using the conventional Holter procedure. Routine follow-up using the Holter procedure and Poincaré plot analysis could help detect early arrhythmias.
|
|
| 10. |
- Rydberg, Annika, et al.
(författare)
-
Serial assessment of variability in heart rate in children with the Fontan circulation.
- 2005
-
Ingår i: Cardiology in the Young. - London : Greenwich Medical Media. - 1047-9511 .- 1467-1107. ; 15:5, s. 498-503
-
Tidskriftsartikel (refereegranskat)abstract
- Autonomic nervous control of the heart can be studied by analysing variability in heart rate. Although earlier studies have shown reduced variability in patients with the Fontan circulation, we are not aware of any previous study examining longitudinal changes in such children. We have examined 13 patients who had undergone total cavopulmonary connection, and 37 healthy controls matched for age and gender. The examinations included complete echocardiography, and 24-hour ambulatory electrocardiogram for analysis of the parameters for variability in heart rate. After the Fontan procedure, three follow-up examinations were performed at a mean of 4.4 years, 5.6 and 7.2 years. Reduced variability was found in those with the Fontan circulation. A significant difference was found between patients and their controls with respect to high-frequency power at the second, p equal to 0.05, and third, p equal to 0.03, examination. The ratio of low-to-high-frequency components progressively increased in those with the Fontan circulation, a phenomenon that led to a significant difference, p equal to 0.03, at the third examination. Our study shows that, in patients with the Fontan circulation, routine ambulatory electrocardiographic monitoring including analysis of variability in heart rate, detects over time a progressive sympatovagal imbalance.
|
|
