| 1. |
- Adrian, Katrin, 1966, et al.
(författare)
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Procedure-induced inflammation and endothelial cell activation in an artificially ventilated and circulated porcine double-lung model
- 2006
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Ingår i: Artif Organs. - 0160-564X. ; 30:12, s. 922-8
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Tidskriftsartikel (refereegranskat)abstract
- Systemic inflammation is induced during extracorporeal circulation, resulting in an increased bleeding tendency and endothelial cell activation. Lungs from seven piglets were perfused by autologous blood in an extracorporeal circuit, where the lungs and the left atrium were attached to polyvinyl chloride (PVC) tubings and the blood circulated by a roller pump. The trachea was intubated and attached to a ventilator. The lungs maintained good gas exchange, despite a slight increase in lactate levels. Plasma tPA increased slightly over time, suggesting endothelial cell activation. Activation of inflammatory systems was reflected in increased levels of plasma interleukin (IL)-6 and IL-10. A model for the study of lung endothelial activation during extracorporeal circulation has been shown to be reproducible. The lung tissue was shown to be capable of gas exchange and activation of endothelial cells and procedure-induced inflammation were noted.
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| 2. |
- Bräutigam, Matilda, 1975, et al.
(författare)
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Linguistic and content validity of the Swedish version of the PedsQL (TM) gastrointestinal symptoms scales and symptoms module for paediatric patients
- 2021
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Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 110:11, s. 3124-3130
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Tidskriftsartikel (refereegranskat)abstract
- Aim To describe the process of linguistic and content validity of the Swedish version of the PedsQL (TM) Gastrointestinal Symptoms Scales and Symptoms Module, measuring health-related quality of life (HRQOL) in children with gastrointestinal (GI) disorders. Methods The establishment of linguistic and content validity was carried out in compliance with international standards on patient-reported outcome measurements. The process included forward translation, expert review and reconciliation, backward translation, backward translation review and interviews with 15 children aged 5-18 years with GI tract symptoms and 20 parents of children with GI tract symptoms aged 2-18 years. Results The Swedish version of the PedsQL (TM) Gastrointestinal Symptoms Scales and Symptoms Module (child report 5-18 years, parent report for children 2-18 years) was achieved without major difficulties. Eight issues needed discussion after forward translation, and there was one change after backward translation and three revisions following patient and parent testing. Conclusion A conceptually equivalent Swedish language version of PedsQL (TM) Gastrointestinal Symptoms Scale and Symptoms Module for children aged 2-18 years old was developed. This enables improved HRQOL evaluations in children with GI disorders in Sweden. Future research using a larger sample is recommended to evaluate validity and reliability of the Swedish language version of the module.
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| 3. |
- Bräutigam, Matilda, 1975, et al.
(författare)
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The Swedish PedsQL gastrointestinal symptoms scale and symptoms module showed good psychometric performance
- 2024
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Ingår i: ACTA PAEDIATRICA. - 0803-5253 .- 1651-2227.
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Tidskriftsartikel (refereegranskat)abstract
- AimThere is no validated symptom scale for Swedish children with gastrointestinal disorders. Our aim was to validate the Swedish version of the Paediatric Quality of Life Inventory (PedsQL) gastrointestinal symptoms scale and symptoms module. MethodsFamilies were recruited from two hospitals in Gothenburg, Sweden, from 1 March 2021 to 31 October 2022. The instruments were completed by 115 children with functional, congenital or organic acquired gastrointestinal disorders and 149 of their parents. These were the gastrointestinal symptoms scales, symptoms module and the 4.0 Generic core scale. Data were analysed for feasibility, construct validity and reliability, including internal consistency, re-test reliability and child-parent agreement. ResultsFeasibility was good, with a failure to respond of <= 5%. Construct validity showed strong correlation in the PedsQL gastrointestinal symptoms module. The known-group validity agreed with the expectations associated with the disease characteristics (p < 0.05). Cronbach's alpha was 0.96, which indicated excellent internal reliability. The intraclass correlation coefficient for the child self-report and parent-proxy report was 0.74, which indicated good agreement. ConclusionThe Swedish PedsQL Gastrointestinal Symptoms Scales, the symptoms module provided acceptable measurement properties and can be used to evaluate symptoms of gastrointestinal disorders and quality of life during clinical work or research projects.
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| 4. |
- Dellenmark-Blom, Michaela, 1983, et al.
(författare)
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Coping strategies used by children and adolescents born with esophageal atresia - a focus group study obtaining the child and parent perspective
- 2016
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Ingår i: Child Care Health and Development. - : Wiley. - 0305-1862. ; 42:5, s. 759-767
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundEsophageal atresia (EA) is a rare malformation, which requires surgical treatment. Survival rates today reach 95%, but EA remains a significant cause of chronic morbidity with increased risk of psychosocial problems and impaired health-related quality of life (HRQOL). No study of coping strategies of children with EA has been reported in the literature to date, but increased knowledge could lead to improved outcomes and better HRQOL. MethodsStandardized focus groups with children with EA and their parents were conducted to identify issues related to health care needs and HRQOL, with group members relating their coping experiences. Identified coping statements were content analysed using a card sorting procedure and descriptive statistics. ResultsThirty families (18 children 8-17years; 32 parents of children with EA 2-17years) participated in 10 focus groups. A total of 590 coping statements were recorded. Nine coping strategies were identified: problem solving (n=116), avoidance (n=95), recognizing responsibility (n=71), confronting (n=70), seeking social support (n=63), positive reappraisal (n=58), emotional expression (n=46), acceptance (n=40) and distancing (n=31). Nine situational contexts were identified: nutritional intake (n=227), communication of one's health condition (n=78), self-perception when experiencing troublesome symptoms (n=59), appearance of body or scar(s) (n=57), physical activities like sport and play (n=43), sleep (n=34), hospital care (n=33), stigmatization and social exclusion (n=30) and medication intake (n=29). ConclusionsFocus group methodology contributed to an increased understanding of disease-specific coping processes among children and adolescence with EA. Findings illustrate that they use several coping strategies, some of which they seem to adopt at early age and use in disease-related contexts of physical, social and emotional character. Such coping may influence health and HRQOL in children with EA. In view of the importance of establishing good coping strategies early in life, health care professionals should integrate coping aspects into care management. Future studies are warranted.
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| 5. |
- Dellenmark-Blom, Maria, 1983, et al.
(författare)
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Factors of family impact in a Swedish-German cohort of children born with esophageal atresia
- 2022
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Ingår i: Orphanet Journal of Rare Diseases. - : Springer Science and Business Media LLC. - 1750-1172. ; 17:1
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Tidskriftsartikel (refereegranskat)abstract
- Background After repair of esophageal atresia (EA), childhood survivors commonly present with digestive and respiratory morbidity, and around 55% have associated anomalies. Although it is known that these problems can reduce health-related quality of life in children with EA, less is understood about the impact on the family. We aimed to identify factors related to family impact in children with EA. Methods One parent each of a child with EA (2-18 years) in 180 families from Sweden and Germany answered the PedsQL (TM) Family Impact Module as the dependent variable. The independent variables were the child's parent-reported health-related quality of life as measured by PedsQL (TM) 4.0, current symptoms, school situation, and parent/family characteristics together with child clinical data from the medical records. Results Stepwise multivariable regression analysis showed a multifactorial model of the total family impact scores (R-2 = 0.60), with independent factors being the child's overall generic health-related quality of life, school-absence >= 1/month, severe tracheomalacia, a family receiving carer's allowance, and a parent with no university/college education, p < 0.05. Logistic regression analysis showed that an increased number of symptoms in the child the preceding 4 weeks lowered the family impact scores; however, the child's feeding (R-2 = 0.35) and digestive symptoms (R-2 = 0.25) explained more in the variation of scores than the child's respiratory symptoms (R-2 = 0.09), p < 0.0001. Conclusions Family functioning may be a contributing factor to the maintenance of child health. The study findings suggest multifactorial explanations to family impact in children with EA, which are essential when optimizing the support to these families in clinical and psychosocial practice. Future research should explore experiences of family impact from all family members' perspectives and multicenter studies are warranted to understand better the effectiveness of psychosocial-educational interventions to families of children with EA.
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| 6. |
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| 7. |
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| 8. |
- Dellenmark-Blom, Michaela, 1983, et al.
(författare)
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Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia: a nationwide Swedish study
- 2022
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Ingår i: Orphanet Journal of Rare Diseases. - : Springer Science and Business Media LLC. - 1750-1172. ; 17:1
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Tidskriftsartikel (refereegranskat)abstract
- Background In 10-15% of children with esophageal atresia (EA) delayed reconstruction of esophageal atresia (DREA) is necessary due to long-gap EA and/or prematurity/low birth weight. They represent a patient subgroup with high risk of complications. We aimed to evaluate postoperative morbidity and health-related quality of life (HRQOL) in a Swedish national cohort of children with DREA. Methods Postoperative morbidity, age-specific generic HRQOL (PedsQL((TM)) 4.0) and condition-specific HRQOL (The EA-QOL questionnaires) in children with DREA were compared with children with EA who had primary anastomosis (PA). Factors associated with the DREA group's HRQOL scores were analyzed using Mann-Whitney U-test and Spearman's rho. Clinical data was extracted from the medical records. Significance level was p < 0.05. Results Thirty-four out of 45 families of children with DREA were included and 30 returned the questionnaires(n = 8 children aged 2-7 years; n = 22 children aged 8-18 years). Compared to children with PA(42 children aged 2-7 years; 64 children aged 8-18 years), there were no significant differences in most early postoperative complications. At follow-up, symptom prevalence in children aged 2-7 with DREA ranged from 37.5% (heartburn) to 75% (cough). Further digestive and respiratory symptoms were present in >= 50%. In children aged 8-18, it ranged from 14.3% (vomiting) to 40.9% (cough), with other digestive and airway symptoms present in 19.0-27.3%. Except for chest tightness (2-7 years), there were no significant differences in symptom prevalence between children with DREA and PA, nor between their generic or condition-specific HRQOL scores (p > 0.05). More children with DREA underwent esophageal dilatations (both age groups), gastrostomy feeding (2-7 years), and antireflux treatment (8-18 years), p < 0.05. Days to hospital discharge after EA repair and a number of associated anomalies showed a strong negative correlation with HRQOL scores (2-7 years). Presence of cough, airway infection, swallowing difficulties and heartburn were associated with lower HRQOL scores (8-18 years), p < 0.05. Conclusions Although children with DREA need more treatments, they are not a risk group for postoperative morbidity and impaired HRQOL compared with children with PA. However, those with a long initial hospital stay, several associated anomalies and digestive or respiratory symptoms risk worse HRQOL. This is important information for clinical practice, families and patient stakeholders.
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| 9. |
- Dellenmark-Blom, Michaela, et al.
(författare)
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Prevalence of Mental Health Problems, Associated Factors, and Health-Related Quality of Life in Children with Long-Gap Esophageal Atresia in Sweden
- 2023
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Ingår i: Journal of Pediatric Surgery. - : Elsevier. - 0022-3468 .- 1531-5037. ; 58:9, s. 1646-1655
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Tidskriftsartikel (refereegranskat)abstract
- Background: Children with long-gap esophageal atresia (LGEA) face a high risk of digestive and respiratory morbidity, but their mental health outcomes have not been investigated. We aimed to identify the prevalence of mental health problems in children with LGEA, associated factors and health-related quality of life (HRQOL).Methods: Twenty-six children with LGEA aged 3-17 were recruited nationwide in Sweden. One of their parents and adolescents aged 11-17 completed information on the child's mental health (Strength and Difficulties Questionnaire), generic (PedsQL 4.0) and condition-specific HRQOL (EA-QOL). Parents gave information on current child symptomatology. Mental health level was determined using validated norms; abnormal >= 90 percentile/borderline >= 80 percentile/normal. Elevated levels were considered borderline/abnormal. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05.Results: Twelve children with LGEA aged 3-17 (46%) had elevated scores of >= 1 mental health domain in parent-reports, whereas 2 adolescents (15%) in self-reports. In parent-reports, 31% of the children had elevated levels of peer relationship problems, with associated factors being child sex male (p = 0.037), airway infections (p = 0.002) and disturbed night sleep (p = 0.025). Similarly, 31% showed elevated levels of hyperactivity/inattention, and associated factors were male sex (p = 0.005), asthma (p = 0.028) and disturbed night sleep (p = 0.036). Elevated levels of emotional symptoms, seen in 20%, were related to swallowing difficulties (p = 0.038) and vomiting problems (p = 0.045). Mental health problems correlated negatively with many HRQOL domains (p < 0.05).Conclusions: Children with LGEA risk mental health difficulties according to parent-reports, especially peer relationship problems and hyperactivity/inattention, with main risk factors being male sex, airway problems and sleep disturbances. This should be considered in follow-up care and research, particularly since their mental health problems may impair HRQOL.
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| 10. |
- Dellenmark-Blom, Michaela, 1983, et al.
(författare)
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Schooling experiences in children with long-gap esophageal atresia compared with children with esophageal atresia and primary anastomosis: a Swedish study
- 2023
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Ingår i: Orphanet Journal of Rare Diseases. - : BioMed Central (BMC). - 1750-1172. ; 18:1
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundChildren with long-gap esophageal atresia (LGEA) risk living with aerodigestive morbidity and mental health difficulties. No previous study has investigated their experiences of schooling, despite the importance of schools in children's development, learning and social relationships. We aimed to describe experiences of schooling in children with LGEA in Sweden in comparison with children with EA who had primary anastomosis.MethodChildren with LGEA aged 3-17 were recruited nationwide in Sweden. One parent completed a survey on their child's school-based supports (according to definitions from the Swedish National Agency for Education), school absence, school satisfaction, school functioning (PedsQL 4.0), mental health (Strength and Difficulties Questionnaire) and current symptomatology. School data were compared between 26 children with LGEA to that from 95 children with EA who had PA, a hypothesized milder affected group. Mental health level was determined using validated norms; abnormal & GE; 90 percentile. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05.ResultsFormal school-based support was reported in 17 (65.4%) children with LGEA and concerned support with nutritional intake (60%), education (50%) and medical/special health needs (35%). The prevalence of school-based support was significantly higher compared to children with PA overall (36.8%, p = 0.013) and regarding nutritional intake support (20%, p < 0.001). In children with LGEA, school-based support was related to low birth weight (p = 0.036), young child age (p = 0.014), height & LE; -2SD for age/sex (p = 0.024) and an increased number of aerodigestive symptoms (p < 0.05). All children with LGEA who had abnormal mental health scores had school-based support, except for one child. Nine children with LGEA (36%) had school absence & GE; 1times/month the past year, more frequently because of colds/airway infections (p = 0.045) and GI-specific problems compared to PA (p = 0.003). School functioning scores were not significantly different from children with PA (p = 0.34) but correlated negatively with school-based support (< 0.001) and school absence (p = 0.002). One parent out of 26 reported their child's school satisfaction as "not good".ConclusionsChildren with LGEA commonly receive school-based support, reflecting multifaceted daily needs and disease severity. School absence is frequent and related to poorer school functioning. Future research focusing on academic achievement in children with EA is needed.
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| 11. |
- Dellenmark-Blom, Michaela, 1983, et al.
(författare)
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The Esophageal-Atresia-Quality-of-life Questionnaires: Feasibility, Validity and Reliability in Sweden and Germany.
- 2018
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Ingår i: Journal of pediatric gastroenterology and nutrition. - 1536-4801. ; 67:4, s. 469-477
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Tidskriftsartikel (refereegranskat)abstract
- Esophageal atresia (EA) is a rare malformation characterized of discontinuity of the esophagus, concurrent with or without a tracheoesophageal fistula (TEF). We report the feasibility validity and reliability of a condition-specific quality-of-life (QOL) tool for EA/TEF children, the age-adapted EA-QOL-questionnaires, when used in Sweden and Germany.A total of 124 families of children with EA/TEF participated in the study; 53 parents completed the EA-QOL-questionnaire for children aged 2 to 7 years; 62 children/71 parents the EA-QOL-questionnaire for children 8 to 17 years. Feasibility was determined from the percentage of missing item responses. Based on clinical data and previously validated generic QOL-instruments (PedsQL 4.0, DISABKIDS-12), the final EA-QOL scores were evaluated against hypotheses of validity (known-groups/concurrent/convergent) and reliability (internal consistency/retest reliability of scores for 3 weeks). Significant level was P < 0.05.In the questionnaire for EA/TEF children aged 2 to 7 years, 16/18 items were completed with missing values <6% (range 0%-7.5%), and in the questionnaire for 8 to 17-year-olds, 24/24 child-reported items (range 0%-4.8%) and 21/24 parent-reported items (range 0%-7.0%). In both age-specific EA-QOL-questionnaires, desirable standards for known-groups and concurrent validity were fulfilled; digestive symptoms and feeding difficulties negatively impacted EA-QOL-Total-scores (P < 0.001), and as hypothesized, in 2 to 7-year-olds, respiratory symptoms decreased EA-QOL-Total-scores (P=0.002). Correlations between the EA-QOL and generic QOL questionnaires supported convergent validity. Internal consistency reliability was satisfactory. The level of agreements of EA-QOL-scores between the field- and retest study were good to excellent.The overall psychometric performance of the EA-QOL-questionnaires for EA/TEF children is satisfactory and can enhance outcome evaluations in future research and clinical practice.
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| 12. |
- Gatzinsky, Vladimir, 1966, et al.
(författare)
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Dysphagia in adults operated on for esophageal atresia--use of a symptom score to evaluate correlated factors.
- 2011
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Ingår i: European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie. - : Georg Thieme Verlag KG. - 1439-359X. ; 21:2, s. 94-8
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Tidskriftsartikel (refereegranskat)abstract
- Dysphagia is not unusual following repair of esophageal atresia (EA). The lack of a uniform definition has led to a variance when it comes to reporting the prevalence of dysphagia among patients operated on for EA. Our aim is to estimate the occurrence and degree of dysphagia, using a numerical score with its statistical versatility independent of a specific definition. The results are used to find early risk factors of dysphagia within this patient group. The results are also used to see whether we can find a correlation between dysphagia and symptoms of gastroesophageal reflux (GER) and quality of life (QoL).
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| 13. |
- Gatzinsky, Vladimir, 1966, et al.
(författare)
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Impaired peripheral airway function in adults following repair of esophageal atresia.
- 2014
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Ingår i: Journal of pediatric surgery. - : Elsevier BV. - 1531-5037 .- 0022-3468. ; 49:9, s. 1347-52
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Tidskriftsartikel (refereegranskat)abstract
- Esophageal atresia (EA) often leads to persistent symptoms and impaired respiratory function in adulthood. The role of peripheral airways in this impairment has not been previously investigated. Furthermore, asthma-like symptoms are common in these patients.
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| 14. |
- Gatzinsky, Vladimir, 1966, et al.
(författare)
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Long-term respiratory symptoms following esophageal atresia.
- 2011
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Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253. ; 100:9, s. 1222-1225
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Tidskriftsartikel (refereegranskat)abstract
- Background: Oesophageal atresia (OA) is a congenital malformation that can lead to persistent respiratory symptoms in adulthood. Aim: To describe the prevalence of respiratory symptoms in adulthood in a population-based study of patients with repaired OA and to compare this with the prevalence in the general population. Methods: Of 80 patients operated for OA in Gothenburg in 1968–1983, 79 were located. The patients received a questionnaire on respiratory symptoms. Controls were 4979 gender- and age-matched subjects who answered the same questions. Results: The questionnaire was answered by 73 of 79 (92%) patients. Physician-diagnosed asthma was reported by 30% in the OA group vs 10% in the control group (OR 4.1; 95% CI 2.4–6.8), and recurrent wheeze in 29% vs 5.5% (OR 6.9; 4.1–11.6). Also wheeze during the last year, asthma medication, a long-standing cough, cough with sputum production and chronic bronchitis were significantly more common among the patients with OA. In contrast, there was no significant difference regarding risk factors for asthma. The prevalence of respiratory symptoms did not appear to decrease with age. Conclusion: A high prevalence of respiratory symptoms remains among adult patients with repaired OA. Many of the patients had an asthma diagnosis. However, asthma heredity or allergic rhinitis was not overrepresented.
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| 15. |
- Gatzinsky, Vladimir, 1966
(författare)
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Respiratory and esophageal morbidity in adults with repaired esophageal atresia
- 2014
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Background: Esophageal atresia (EA) often leads to persistent esophageal and respiratory symptoms, as well as impaired esophageal and lung function in adulthood. The reasons for this, and the connections between symptoms and documented abnormalities, are not fully understood. Purpose: We wanted to investigate a cohort of adults previously operated on for EA in order to describe the prevalence of symptoms and functional abnormalities, as well as to investigate whether, and if so how, they are connected. Methods: Seventy-three of 79 patients operated on for EA in Gothenburg in 1968-1983 agreed to participate in the first part of the study involving questionnaires relating to symptoms from the esophagus and respiratory tract. Twenty-nine patients agreed to undergo further investigations of pulmonary and esophageal function. Results: From the questionnaire studies, we found that, even though the overall quality of life was good, a considerable number of patients had troublesome symptoms. Both esophageal and respiratory symptoms were frequent. Fifty-seven percent experienced swallowing disturbances (dysphagia) which appeared to be associated with regurgitation, which was in turn noted in 40%. Thirty-two percent experienced heartburn. Different respiratory symptoms, such as wheeze and long-standing cough (44% and 30% respectively), were much more common in this cohort than in the general population. Asthma was reported by 30%, even though no predisposing factors were noted. Impaired respiratory function, either obstructive and/or restrictive, was noted in 22/28 (79%). The obstruction was mainly in the peripheral airways, 17/28 (61%) subjects (measured by multiple-breath inert gas washout, MBW), while only six (21%) subjects displayed values indicating central obstruction. Nine patients had restrictive disease. Airway hyper-responsiveness was frequent and associated with atopy and airway inflammation. However, respiratory symptoms or doctor-diagnosed asthma (DDA) did not correlate with any specific lung function test abnormality. There was a high prevalence of gastro-esophageal reflux (GER) measured by pH multichannel intraluminal impedance (pH-MII) involving both pathological reflux episodes with a pH of < 4 and of > 4 (5/15 and 10/15 subjects respectively). Dysphagia correlated to the number of weakly acidic reflux episodes, while esophageal mucosal damage (14/24 subjects with esophagitis, two of whom had Barrett’s esophagus) correlated to the reflux index (RI) and the number of episodes of weakly acidic reflux. Lower esophageal sphincter incompetence to any extent was frequent (21/24 subjects) and correlated to the number of acid reflux episodes and RI. Conclusion: A high prevalence of both respiratory and esophageal symptoms remains in adulthood. The impaired pulmonary function appears to be more pronounced than previously described. Even non-acidic reflux episodes appear to contribute to the esophageal morbidity. New investigative modalities such as MBW and pH-MII have helped us in further describing and understanding the late sequelae of EA. Classical asthma appears to be difficult to diagnose in this patient group. Given the high prevalence of both respiratory and esophageal morbidity, further studies and long-term follow-up, including MBW and pH-MII, are warranted.
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| 16. |
- Hukkinen, Maria, et al.
(författare)
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Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries
- 2020
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Ingår i: HPB. - : Elsevier BV. - 1365-182X. ; 22:1, s. 161-168
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Tidskriftsartikel (refereegranskat)abstract
- Background: Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear. Methods: Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices. Results: During 2000–2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46–5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0–7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13–1.54, p < 0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported. Conclusions: CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged.
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| 17. |
- Jacobsen, Ronni Bengtson, et al.
(författare)
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Ultra-high-frequency ultrasound (48-70 MHz) is a promising tool for improved gastrointestinal diagnostics in infants
- 2024
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Ingår i: ACTA PAEDIATRICA. - 0803-5253 .- 1651-2227.
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Tidskriftsartikel (refereegranskat)abstract
- Aim: To investigate the clinical feasibility of ultra-high-frequency abdominal ultrasound (UHFUS) scans of preterm and term infants. Methods: Prospectively, 19 healthy term newborn infants were examined with conventional ultrasound (CUS) (Toshiba, Aplio i700, linear probe 14L5) and UHFUS (Visualsonics VevoMD, linear probes UHF48 and UHF70) according to a standardised protocol. Measurements of wall thickness were performed for; stomach, small intestine, colon and peritoneum. Five preterm infants, with or without suspected necrotising enterocolitis (NEC), were also examined with UHF48. Of these, only one was later diagnosed with NEC. Results: Differences between CUS and UHFUS (UHF48) were found in measurements of thickness; for peritoneum 0.25 versus 0.13 mm (p < 0.001), small intestine 0.76 versus 0.64 mm (p = 0.039) and colon 0.7 versus 0.47 mm (p < 0.001) in healthy term infants. Gaining frequency from 46 to 71 MHz showed a mean reduction in measurements of peritoneum from 0.13 to 0.09 mm (p < 0.001). One preterm infant with NEC showed a fivefold and twofold increase in peritoneal and gastrointestinal wall thickness respectively, compared to healthy preterm infants. Conclusion: UHFUS was a clinically feasible, promising method with potential to improve gastrointestinal diagnostics in infants. Lower peritoneum thickness and gastrointestinal wall thickness were demonstrated with UHFUS compared to CUS, suggesting an overestimation by CUS.
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| 18. |
- Jönsson, Linus, 1973, et al.
(författare)
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Early Regenerative Response in the Intrathoracic Porcine Esophagus-The Impact of the Inflammation.
- 2014
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Ingår i: Artificial Organs. - : Wiley. - 0160-564X. ; 38:6, s. 439-446
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Tidskriftsartikel (refereegranskat)abstract
- In order to improve the treatment of children born with long-gap esophageal atresia, a porcine model was developed for studying esophageal regrowth using a bridging graft composed of a silicone stented Biodesign mesh. The aim of the study was to investigate how leakage and contact between the native muscle and Biodesign mesh affected the early healing response. Resection of 3cm of intrathoracic esophagus was performed in 10 newly weaned piglets. They were fed through a gastrostomy 8-10 days prior to sacrifice. In order to achieve nonleaking anastomoses, the silicone stent and suturing technique had to be adjusted between the first four and second six piglets. The technical adjustment decreased leakage. A nonleaking anastomosis could not be achieved when the native muscle layers were sewn less central on the bridging graft compared with the mucosa. If there was leakage, the inflammatory response increased, with islets of perivascular T-lymphocytes and infiltration of macrophages in the native muscle layers. In the bridging area, new vessels were seen in the submucosa in 9 of 10 piglets between 4 and 10 days after surgery. Smooth muscle cells also appeared to move from the cut muscle edges of both the muscularis mucosa and the lamina muscularis and were seen as a layer of several cells under newly formed mucosa. Double staining of the basal membrane of the ingrowing vessels and the pericytes showed that the basal membrane was thinner over some of the pericytes, but there was no accumulation of immature-looking cells in the submucosa of the bridging area. In this porcine model, where esophageal regrowth was studied by using a bridging graft composed of a silicone stented Biodesign mesh, we can conclude that leakage increased the inflammatory response in early healing. Ingrowth of new vessels was seen in the bridging area and movement of smooth muscle cells was found under newly formed mucosa.
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| 19. |
- Jönsson, Linus, 1973, et al.
(författare)
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Macrophage Phenotype Is Associated With the Regenerative Response in Experimental Replacement of the Porcine Esophagus.
- 2016
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Ingår i: Artificial organs. - : Wiley. - 1525-1594 .- 0160-564X. ; 40:10, s. 950-958
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Tidskriftsartikel (refereegranskat)abstract
- A porcine model for bridging circumferential defects in the intrathoracic esophagus has been developed in order to improve the treatment of children born with long-gap esophageal atresia. The aim of this study was to identify factors beneficial for tissue regeneration in the bridging area in this model and to describe the histological progression 20 days after replacement with a silicone-stented Biodesign mesh. Resection of 3cm of intrathoracic esophagus and replacement with a bridging graft was performed in six newly weaned piglets. They were fed through a gastrostomy for 10 days, and then had probe formula orally for another 10 days prior to sacrifice. Two out of six piglets had stent loss prior to sacrifice. In the four piglets with the stent in place, a tissue tube, with visible muscle in the wall, was seen at sacrifice. Histology showed that the wall of the healing area was well organized with layers of inflammatory cells, in-growing vessels, and smooth muscle cells. CD163+ macrophages was seen toward the esophageal lumen. In the animals where the stent was lost, the bridging area was narrow, and histology showed a less organized structure in the bridging area without the presence of CD163+ macrophages. This study indicates that regenerative healing was seen in the porcine esophagus 20 days after replacement of a part of the intrathoracic esophagus with a silicone-stented Biodesign mesh, if the bridging graft is retained. If the graft is lost, the inflammatory pattern changes with invasion of proinflammatory, M1 macrophages in the entire wall, which seems to redirect the healing process toward scar formation.
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| 20. |
- Jönsson, Linus, 1973, et al.
(författare)
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Piglet model for studying esophageal regrowth after resection and interposition of a silicone stented small intestinal submucosa tube.
- 2011
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Ingår i: European surgical research. Europäische chirurgische Forschung. Recherches chirurgicales européennes. - : S. Karger AG. - 1421-9921. ; 46:4, s. 169-79
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: To investigate the use and subsequent healing of a silicone stented small intestinal submucosa (SIS) tube as a full-circumference replacement following surgical resection of the esophagus in piglets. Material and Methods: Three centimeters of the intrathoracic esophagus was replaced with a silicone stented SIS tube (Cook Medical) in 6 growing piglets. The esophageal stent was retained for 4 weeks. Esophageal dilations were performed, if needed, after stent removal. Results: The piglets were sacrificed 1-17 weeks after surgery. Recurrent dilations were needed after stent removal. Histology showed that the gap between the resection margins was filled with new loose connective tissue consisting of fibroblasts and few inflammatory cells. In this tissue, intense angiogenesis was seen at the early time points, which then gave way to the proliferation of immature-looking smooth-muscle-like cells in the submucosa, which appeared to stem from the pericytes of the ingrowing capillaries. Conclusions: Through using a stented SIS tube as a circumferential esophageal replacement in a piglet model, this study suggests that pericytes from ingrowing capillaries may play a role in the remodeling of the SIS mesh. It remains to be seen if this process gives a favorable end result because stricture formation after stent removal remains a problem.
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| 21. |
- Pakarinen, Mikko P, et al.
(författare)
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Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016
- 2018
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Ingår i: Journal of Pediatric Surgery. - : Elsevier BV. - 0022-3468 .- 1531-5037. ; 53:8, s. 1509-1515
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Tidskriftsartikel (refereegranskat)abstract
- Background/purpose: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. Methods: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016. Results: Of the 154 patients, 148 underwent portoenterostomy mostly by assigned surgical teams at median age of 64 (interquartile range 37-79) days, and 95 patients (64%) normalized their serum bilirubin concentration while living with native liver. Postoperative adjuvant medical therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >. 3 patients and diagnostic protocol without routine liver biopsy predicted early performance of portoenterostomy. The cumulative 5-year native liver and overall survival estimate was 53% (95% CI 45-62) and 88% (95% CI 83-94), respectively. Portoenterostomy age <. 65. days and annual center caseload >. 3 patients were predictive for long-term native liver survival, while normalization of serum bilirubin after portoenterostomy was the major predictor of both native liver and overall 5-year survival. Conclusions: The outcomes of biliary atresia in the Nordic countries compared well with previous European studies. Further improvement should be pursued by active measures to reduce patient age at portoenterostomy. Retrospective prognosis study: Level II.
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| 22. |
- Witt, Stefanie, et al.
(författare)
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Health-related quality of life experiences in children and adolescents born with esophageal atresia: A Swedish-German focus group study.
- 2019
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Ingår i: Child: care, health and development. - : Wiley. - 1365-2214 .- 0305-1862. ; 45:1, s. 79-88
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Tidskriftsartikel (refereegranskat)abstract
- Esophageal atresia (EA) is a rare malformation of the esophagus, which needs surgical treatment. Survival rates have reached 95%, but esophageal and respiratory morbidity during childhood is frequent. Child and parent perspectives and cultural and age-specific approaches are fundamental in understanding children's health-related quality of life (HRQoL) and when developing a pediatric HRQoL questionnaire. We aimed to increase the conceptual and cross-cultural understanding of condition-specific HRQoL experiences among EA children from Sweden and Germany and investigate content validity for an EA-specific HRQoL questionnaire.Eighteen standardized focus groups (FGs) with 51 families of EA children aged 2-17years in Sweden (n=30 families) and Germany (n=21 families) were used to explore HRQoL experiences, which were content analyzed into HRQoL domains. The Swedish HRQoL domains were analyzed first and used as framework to evaluate HRQoL content reported in the German FGs. HRQoL experiences were then categorized as physical, social, and emotional HRQoL burden or resource.One thousand nine hundred eight HRQoL statements were recorded. All nine EA-specific HRQoL domains identified in the Swedish FGs (eating, social relationships, general life issues, communication, body issues, bothersome symptoms, confidence, impact of medical treatment, and additional difficulties due to concomitant anomalies) were recognized in the FGs held in Germany, and no additional EA-specific HRQoL domain was found. The HRQoL dimensions referenced physical burden (n=655, 34.5%), social burden (n=497, 26.0%), social resources (n=303, 15.9%), emotional burden (n=210, 11.0%), physical resources (n=158, 8.3%), and emotional resources (n=85, 4.5%).This first international FG study to obtain the EA child and his or her parents' perspective on HRQoL suggests Swedish-German qualitative comparability of the HRQoL domains and content validity for a cross-cultural EA-specific HRQoL questionnaire. EA children make positive and negative HRQoL experiences, but prominently related to physical and social burden, which underlines appropriate follow-up care and future research.
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| 23. |
- Witt, Stefanie, et al.
(författare)
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Parent-child-agreement on health-related quality of life and its determinants in patients born with Esophageal Atresia: a Swedish-German cross-sectional study.
- 2021
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Ingår i: Orphanet journal of rare diseases. - : Springer Science and Business Media LLC. - 1750-1172. ; 16:1
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Tidskriftsartikel (refereegranskat)abstract
- The aim was to compare parent and child-reported health-related quality of life (HRQOL) of children born with esophageal atresia (EA) and determine factors that affect the level of parent-child agreement.We included 63 parent-child dyads of children born with EA aged 8-18 from Germany and Sweden. The generic PedsQL 4.0™ questionnaire and the condition-specific EA QOL questionnaire were used to assess children's HRQOL from parents' and children's perspectives. The PedsQL™ Family Impact Module was used to assess parental HRQOL and Family Functioning.On an individual level, intra-class correlation coefficients indicated strong levels of parent-child agreement (.61-.97). At the group level, the analyses showed no significant differences between the responses of parents and children. When a disagreement occurred, parents were more likely to rate generic HRQOL lower than the children (19-35%) and condition-specific HRQOL higher than the children (17-33%). Findings of the binary logistic regression analyzes showed that the child's age, gender, and country (Germany vs. Sweden) were significant predictors of parent-child agreement in condition-specific HRQOL. We did not identify any significant variables that explain agreement for the generic HRQOL.The parent-child agreement is mostly good, suggesting that parent-reports are a reliable source of information. However, discrepancies may occur and can be explained by the child's age, gender, and country (Sweden vs. Germany). Both perspectives are essential sources for treating EA patients and should not be considered right or wrong. Instead, this information broadens the perspective on pediatric EA patients.
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| 24. |
- Örnö Ax, Sofie, et al.
(författare)
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The association of feeding difficulties and generic health-related quality of life among children born with esophageal atresia
- 2023
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Ingår i: Orphanet Journal of Rare Diseases. - 1750-1172. ; 18:1
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundChildren born with esophageal atresia experience feeding difficulties. This study investigates the association of feeding difficulties and generic health-related quality of life among children aged 2-7 and 8-17 years, born with esophageal atresia.Methods108 families (n = 36 aged 2-7 years; n = 72 aged 8-17) answered a survey regarding difficulties in their child's mealtimes and a validated generic health-related quality of life instrument(PedsQL 4.0). Clinical data was collected from hospital records. The association of feeding difficulties and health-related quality of life was analysed trough Mann-Whitney U-test. Linear regression determined whether the number of concurrent feeding difficulties in the child decreased the health-related quality of life scores. P < 0.05 was considered significant.ResultsIn children aged 2-7 and 8-17 years, to have a gastrostomy, to use a food infusion pump, need for energy-enriched food and eating small portions were respectively significantly associated with lower total health-related quality of life scores in the parent-reports (p < 0.05). Most of the feeding difficulties had a negative significant relationship with the domains of physical and social functioning. Additionally, in the older age group, long mealtimes and adult mealtime supervision were associated with lower scores in both child and parent reports. In both age groups, an increased number of feeding difficulties in the child decreased the total generic health-related quality of life scores (p < 0.01).ConclusionSpecific feeding difficulties are associated with low health-related quality of life among children with esophageal atresia. An increasing number of feeding difficulties is associated to decreasing health-related quality of life-scores. Further research is needed to understand these associations.
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