| 1. |
- Eckhardt, CL, et al.
(author)
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Factor VIII gene (F8) mutation and risk of inhibitor development in nonsevere hemophilia A
- 2013
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In: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 122:11, s. 1954-1962
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Journal article (peer-reviewed)abstract
- The inhibitor incidence in nonsevere hemophilia A patients with certain F8 mutations approaches the inhibitor incidence in severe patients. These findings are highly relevant for clinical practice, as they facilitate identification of high-risk patients based on F8 genotype.
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| 2. |
- Granlund, P. A., et al.
(author)
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Survey of 23 Nordic university hospitals showed that 77% lacked written procedures for measuring and interpreting blood pressure in infants
- 2019
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In: Acta Paediatrica. - : Wiley. - 0803-5253. ; 108:2, s. 266-270
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Journal article (peer-reviewed)abstract
- Aim This study determined the use of standardised procedures for infant noninvasive blood pressure (NIBP) measurements in the Nordic countries and aimed to identify factors included in the standardisation and interpretation of NIBP measurements in infants. Methods A cross-sectional electronic questionnaire survey was sent to 84 physicians in all 23 university hospitals in Sweden, Norway, Denmark, Finland and Iceland and was completed from February to March 2017. The survey contained respondent characteristics, the presence and description of standardised procedures for NIBP measurements, daily practice of NIBP measurements and methodological considerations and interpretation of NIBP measurements in a healthy six-month-old child. Results We received responses from 55 of 84 physicians working in all 23 Nordic university hospitals, in paediatric cardiology (n = 22), general paediatrics (n = 16), paediatric nephrology (n = 14) and other fields (n = 3). Less than a quarter (23%) said their hospital issued specific NIBP procedures relating to infants and they referred to 19 different sources of information. The factors that were most commonly assessed for interpretation were age (100%), arousal state (78%) and cuff size (76%). Conclusion Most of the university hospital units treating children lacked age-specific written procedures for measuring and interpreting infant NIBP, and there is a strong need for common Nordic guidelines.
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| 3. |
- Jung, Christian, et al.
(author)
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A comparison of very old patients admitted to intensive care unit after acute versus elective surgery or intervention
- 2019
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In: Journal of critical care. - : W B SAUNDERS CO-ELSEVIER INC. - 0883-9441 .- 1557-8615. ; 52, s. 141-148
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Journal article (peer-reviewed)abstract
- Background: We aimed to evaluate differences in outcome between patients admitted to intensive care unit (ICU) after elective versus acute surgery in a multinational cohort of very old patients (80 years; VIP). Predictors of mortality, with special emphasis on frailty, were assessed.Methods: In total, 5063 VIPs were induded in this analysis, 922 were admitted after elective surgery or intervention, 4141 acutely, with 402 after acute surgery. Differences were calculated using Mann-Whitney-U test and Wilcoxon test. Univariate and multivariable logistic regression were used to assess associations with mortality.Results: Compared patients admitted after acute surgery, patients admitted after elective surgery suffered less often from frailty as defined as CFS (28% vs 46%; p < 0.001), evidenced lower SOFA scores (4 +/- 5 vs 7 +/- 7; p < 0.001). Presence of frailty (CFS >4) was associated with significantly increased mortality both in elective surgery patients (7% vs 12%; p = 0.01), in acute surgery (7% vs 12%; p = 0.02).Conclusions: VIPs admitted to ICU after elective surgery evidenced favorable outcome over patients after acute surgery even after correction for relevant confounders. Frailty might be used to guide clinicians in risk stratification in both patients admitted after elective and acute surgery.
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| 4. |
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| 5. |
- Lövdahl, Susanna, et al.
(author)
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A longitudinal study of family structure in Swedish persons with haemophilia
- 2014
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In: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 20:4, s. 493-499
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Journal article (peer-reviewed)abstract
- Haemophilia is an X-linked inherited rare bleeding disorder affecting mainly men. The treatment consists of replacement therapy that has been associated with severe side effects, such as blood transmitted viral infections, but has markedly improved over the last decades. The aim of this study was to study family structure over time among Swedish persons with haemophilia (PWH), focusing on children, siblings and marital status. PWH A or B were identified from the haemophilia centres and the national Patient Registry. Each PWH was compared to five age- and gender-matched controls. The national Multi-Generation Registry was used to identify children and siblings. A total of 1365 children with a father suffering from haemophilia A or B and 1938 siblings of the PWH were identified. Having one or more children was significantly less common (P=0.003) for PWH than for controls. Significantly lower rates of having a child were also found for the subgroups of persons suffering from severe haemophilia and those infected with HIV (P<0.001). A higher proportion of PWH, with or without HIV and/or viral hepatitis had siblings compared to the controls (P<0.001). However, the mean number of siblings was significantly lower for persons with severe haemophilia (P=0.001). The number of marriages and divorces did not differ between PWH and controls. Our data indicate a negative impact of HIV and viral hepatitis on family structure for PWH despite the relatively good access to treatment in Sweden over the last few decades. This was particularly true for those with a severe form of haemophilia.
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| 6. |
- Lövdahl, Susanna, et al.
(author)
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Hypertension and cardiovascular diseases in Swedish persons with haemophilia - A longitudinal registry study
- 2019
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In: Thrombosis Research. - : Elsevier BV. - 0049-3848 .- 1879-2472. ; 181, s. 106-111
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Journal article (peer-reviewed)abstract
- Introduction: Data on the prevalence of hypertension and cardiovascular diseases (CVD) among persons with haemophilia (PWH) vary. Sweden has a long tradition of maintaining population-based data registries, and there is extensive follow-up of haemophilia patients due to the use of prophylaxis over decades. We evaluated the prevalence of these diseases among Swedish PWH compared to matched controls using a longitudinal study design. Methods: Data were obtained from the National Patient Registry and linked to records of persons with haemophilia enrolled in the haemophilia centres. For each subject, five gender and age matched controls were identified. Results: We identified 193 (19.7%) diagnoses of hypertension in PWH born in 1978 or earlier over >= 30 years compared with 550 (11.2%) among controls. The median ages and interquartile ranges were 60.0 (42.8, 69.9) and 57.2 (42.6, 70.6) years. The hazard rate (HR) for hypertension, PWH vs. controls, was 2.1, 95% CI: [1.8; 2.5], p < 0.001. The findings were similar in subgroup analyses of patients with non-severe and severe haemophilia with or without HIV and/or viral hepatitis. Angina pectoris was diagnosed in 69 (4.8%) of patients censored at age 75 compared with 311 (4.3%) in controls, and myocardial ischemia in 84 (5.9%) compared with 442 (6.2%). As a cause of death, the HR for myocardial ischemia, comparing PWH and controls, was 0.58, 95% CI: [0.42, 0.80], p = 0.001. Conclusion: Our data support an increased prevalence of hypertension among persons with haemophilia. The prevalence of CVD seems to be similar to that of controls, but with lower mortality.
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| 7. |
- Osooli, Mehdi, et al.
(author)
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Joint comorbidities among Swedish carriers of haemophilia: A register-based cohort study over 22 years
- 2019
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In: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 25:5, s. 845-850
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Journal article (peer-reviewed)abstract
- Background A significant fraction of women with an impaired factor VIII or IX gene in the X chromosome, carriers of haemophilia, will have clotting factor activities corresponding to those seen in males with non-severe haemophilia, hence, experience an increased bleeding tendency. Data describing the long-term joint outcomes among carriers are limited. We compared the age at onset, frequency of joint-related diagnoses as well as joint surgery and related hospitalizations among carriers of haemophilia with sex- and birthdate-matched controls from the general population. Methods Carriers of haemophilia born 1941-2008 were identified through the haemophilia treatment centres' (HTCs) databases and the National Patient Register of Sweden. For each carrier, we included up to five individuals using the Swedish population register as comparisons. Data for the period 1987-2008 were obtained. Results Among 539 potential carriers identified, 213 had a known factor activity. Carriers with reduced factor activity and those with unknown factor activity had received their first joint-related diagnosis at a significantly earlier age than their comparisons. The same subgroups showed an overall 2.3- and 2.4-fold higher hazard for joint-related diagnoses compared with the general population. In addition, the hazards of joint-related outpatient hospitalization were 3.2-fold (95% CI: 1.2, 9.1) and 2.5-fold (95% CI: 1.6, 3.7). This was not observed for those with normal factor activity. Conclusion Carriers of haemophilia suffer a significant risk for joint comorbidities. This risk seems to correlate to the factor activity. Our findings underline the importance of regular clinical follow-up of carriers at HTCs.
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| 8. |
- Petropoulos, Fotios, et al.
(author)
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Operational Research : methods and applications
- 2024
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In: Journal of the Operational Research Society. - : Taylor & Francis Group. - 0160-5682 .- 1476-9360. ; 75:3, s. 423-617
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Research review (peer-reviewed)abstract
- Throughout its history, Operational Research has evolved to include methods, models and algorithms that have been applied to a wide range of contexts. This encyclopedic article consists of two main sections: methods and applications. The first summarises the up-to-date knowledge and provides an overview of the state-of-the-art methods and key developments in the various subdomains of the field. The second offers a wide-ranging list of areas where Operational Research has been applied. The article is meant to be read in a nonlinear fashion and used as a point of reference by a diverse pool of readers: academics, researchers, students, and practitioners. The entries within the methods and applications sections are presented in alphabetical order. The authors dedicate this paper to the 2023 Turkey/Syria earthquake victims. We sincerely hope that advances in OR will play a role towards minimising the pain and suffering caused by this and future catastrophes.
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| 9. |
- Rasooli, Reza, et al.
(author)
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In Silico Evaluation of a Self-powered Venous Ejector Pump for Fontan Patients.
- 2023
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In: Cardiovascular engineering and technology. - : Springer Science and Business Media LLC. - 1869-408X .- 1869-4098. ; 14:3, s. 428-446
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Journal article (peer-reviewed)abstract
- The Fontan circulation carries a dismal prognosis in the long term due to its peculiar physiology and lack of a subpulmonic ventricle. Although it is multifactorial, elevated IVC pressure is accepted to be the primary cause of Fontan's high mortality and morbidity. This study presents a self-powered venous ejector pump (VEP) that can be used to lower the high IVC venous pressure in single-ventricle patients.A self-powered venous assist device that exploits the high-energy aortic flow to lower IVC pressure is designed. The proposed design is clinically feasible, simple in structure, and is powered intracorporeally. The device's performance in reducing IVC pressure is assessed by conducting comprehensive computational fluid dynamics simulations in idealized total cavopulmonary connections with different offsets. The device was finally applied to complex 3D reconstructed patient-specific TCPC models to validate its performance.The assist device provided a significant IVC pressure drop of more than 3.2 mm Hg in both idealized and patient-specific geometries, while maintaining a high systemic oxygen saturation of more than 90%. The simulations revealed no significant caval pressure rise (< 0.1 mm Hg) and sufficient systemic oxygen saturation (> 84%) in the event of device failure, demonstrating its fail-safe feature.A self-powered venous assist with promising in silico performance in improving Fontan hemodynamics is proposed. Due to its passive nature, the device has the potential to provide palliation for the growing population of patients with failing Fontan.
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| 10. |
- Rasooli, Reza, et al.
(author)
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In vitro hemodynamic performance of a blood pump for self-powered venous assist in univentricular hearts
- 2024
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In: SCIENTIFIC REPORTS. - 2045-2322. ; 14:1
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Journal article (peer-reviewed)abstract
- Univentricular heart anomalies represent a group of severe congenital heart defects necessitating early surgical intervention in infancy. The Fontan procedure, the final stage of single-ventricle palliation, establishes a serial connection between systemic and pulmonary circulation by channeling venous return to the lungs. The absence of the subpulmonary ventricle in this peculiar circulation progressively eventuates in failure, primarily due to chronic elevation in inferior vena cava (IVC) pressure. This study experimentally validates the effectiveness of an intracorporeally-powered venous ejector pump (VEP) in reducing IVC pressure in Fontan patients. The VEP exploits a fraction of aortic flow to create a jet-venturi effect for the IVC, negating the external power requirement and driveline infections. An invitro Fontan mock-up circulation loop is developed and the impact of VEP design parameters and physiological conditions is assessed using both idealized and patient-specific total cavopulmonary connection (TCPC) phantoms. The VEP performance in reducing IVC pressure exhibited an inverse relationship with the cardiac output and extra-cardiac conduit (ECC) size and a proportional relationship with the transpulmonary pressure gradient (TPG) and mean arterial pressure (MAP). The ideal VEP with fail-safe features provided an IVC pressure drop of 1.82 +/- 0.49, 2.45 +/- 0.54, and 3.12 +/- 0.43 mm Hg for TPG values of 6, 8, and 10 mm Hg, respectively, averaged over all ECC sizes and cardiac outputs. Furthermore, the arterial oxygen saturation was consistently maintained above 85% during full-assist mode. These results emphasize the potential utility of the VEP to mitigate elevated venous pressure in Fontan patients.
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