The Experts below are selected from a list of 273 Experts worldwide ranked by ideXlab platform
Hugo Devlieger - One of the best experts on this subject based on the ideXlab platform.
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Agenesis of corpus callosum and anophthalmia in the Asplenia syndrome. A recognisable association
Annales de genetique, 1997Co-Authors: Koenraad Devriendt, Gunnar Naulaers, Gert Matthijs, K Van Houdt, Hugo Devlieger, Marc Gewillig, Jean-pierre FrynsAbstract:We present a newborn infant with the Asplenia syndrome and unique associated features of corpus callosum agenesis, anophthalmia and coloboma. Previous reports of eye abnormalities or corpus callosum agenesis in patients with Asplenia suggest that this may represent a distinct clinically recognisable entity of abnormal lateralisation.
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Asplenia syndrome and isolated total anomalous pulmonary venous connection in siblings.
European journal of pediatrics, 1994Co-Authors: Koenraad Devriendt, Marc Gewillig, A. Casaer, A. Van Cauter, F. De Zegher, Monique Dumoulin, Hugo DevliegerAbstract:We report on a family with Asplenia syndrome in one and total anomalous pulmonary venous connection (TAPVC) in the other sib. Both conditions are rare, may have a genetic cause and belong to a spectrum of laterality disorders. This suggests that both Asplenia syndrome and TAPVC in this family are the clinical expression of a single genetic disorder.
Penelope Jones - One of the best experts on this subject based on the ideXlab platform.
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a registry for patients with Asplenia hyposplenism reduces the risk of infections with encapsulated organisms
Clinical Infectious Diseases, 2018Co-Authors: Alicia Arnott, Penelope Jones, Lucinda J Franklin, Denis Spelman, Karin Leder, Allen C ChengAbstract:Background Overwhelming post-splenectomy infection (OPSI) is a serious complication of Asplenia. Clinical guidelines recommend numerous measures to reduce the risk of OPSI, but awareness and adherence to preventative measures are generally poor. We aimed to determine whether a registry for asplenic/hyposplenic patients was associated with a reduction in the incidence of infection with encapsulated bacteria. Methods We performed a retrospective cohort study of asplenic/hyposplenic patients in the state of Victoria, Australia, who registered with Spleen Australia from 2003 through 2014. Spleen Australia provides education, clinical guidance, and annual vaccination reminders to registrants and their healthcare providers. We compared the incidence of infection with Streptococcus pneumoniae, Haemophilus influenzae type B (Hib), and Neisseria meningitidis before and after registration. Registry data were linked with Victorian notifiable disease data on invasive pneumococcal disease (IPD), invasive meningococcal disease (IMD), and Hib between 2000 and 2014. Results Twenty-seven cases of IPD and 1 of IMD occurred among 3221 registrants. No cases of Hib were reported. The rate of IPD/IMD was 150 per 100000 patient-years prior to registration and 36 per 100000 patient-years after registration; registration was associated with a 69% reduction in the risk of infection (incidence rate ratio, 0.31; 95% confidence interval, 0.12 to 0.83; P = .019). Based on the absolute reduction in incidence, we estimate that Spleen Australia prevents 5-6 invasive infections with encapsulated organisms annually among registrants. Conclusions Systematic, long-term approaches to post-splenectomy care can significantly reduce the risk of infection with encapsulated organisms among individuals with Asplenia/hyposplenism.
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overwhelming post splenectomy sepsis in patients with Asplenia and hyposplenia a retrospective cohort study
Epidemiology and Infection, 2017Co-Authors: Joash Wen Chen Chong, Penelope Jones, Denis Spelman, Karin Leder, Allen C ChengAbstract:Overwhelming post-splenectomy infection (OPSI) is a serious complication of Asplenia and is associated with encapsulated organisms, most commonly Streptococcus pneumoniae, but also Haemophilus influenzae and Neisseria meningitidis. We aimed to estimate the risk of infection in this patient group. We reviewed data collected by the Victorian Spleen Registry in Australia. On registration, all patients are asked about significant infections requiring admission to hospital for intravenous antibiotics; those requiring admission to ICU were defined as OPSI. In the 3274 asplenic patients registered 492 patients reported at least one episode of infection. There were 47 episodes of OPSI requiring intensive care (incidence rate 1·11/1000 patient-years). The risk of OPSI was highest in older patients, and there were no statistically significant differences in incidence by reason for splenectomy except for a higher rate in patients with medical hyposplenia. This study reinforces that post-splenectomy infection is a clinically significant but uncommon complication, and that fulminant infection requiring intensive care is a minority of all infections.
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D: Splenectomy associated changes in IgM memory B cells in an adult spleen registry cohort. PLoS One 2011, 6:e23164. doi:10.1186/1743-422X-9-273 Cite this article as: Tian et al.: Effect of antiviral therapy on the survival and incidence of major com
2016Co-Authors: Paul U. Cameron, Penelope Jones, Malgorzata Gorniak, Kate Dunster, Eldho Paul, Ian Woolley, Denis SpelmanAbstract:Asplenic patients have a lifelong risk of overwhelming post-splenectomy infection and have been reported to have low numbers of peripheral blood IgM memory B cells. The clinical value of quantitation of memory B cells as an indicator of splenic abnormality or risk of infection has been unclear. To assess changes in B cell sub-populations after splenectomy we studied patients recruited to a spleen registry (n = 591). A subset of 209 adult asplenic or hyposplenic subjects, and normal controls (n = 140) were tested for IgM memory B cells. We also determined a) changes in IgM memory B cells with time after splenectomy using the cross-sectional data from patients on the registry and b) the kinetics of changes in haematological markers associated with splenectomy(n = 45). Total B cells in splenectomy patients did not differ from controls, but memory B cells, IgM memory B cells and switched B cells were significantly (p,0.001) reduced. The reduction was similar for different indications for splenectomy. Changes of Asplenia in routine blood films including presence of Howell-Jolly bodies (HJB), occurred early (median 25 days) and splenectomy associated thrombocytosis and lymphocytosis peaked by 50 days. There was a more gradual decrease in IgM memory B cells reaching a stable level within 6 months after splenectomy. IgM memory B cells as proportion of B cells was the best discriminator between splenectomized patients and normal controls and at the optimal cut-off of 4.53, showed a true positive rate of 95 % and false positive rate of 20%. In a survey of 152 registry patient
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splenectomy associated changes in igm memory b cells in an adult spleen registry cohort
PLOS ONE, 2011Co-Authors: Penelope Jones, Paul U. Cameron, Malgorzata Gorniak, Kate Dunster, Eldho Paul, Sharon R Lewin, Ian WoolleyAbstract:Asplenic patients have a lifelong risk of overwhelming post-splenectomy infection and have been reported to have low numbers of peripheral blood IgM memory B cells. The clinical value of quantitation of memory B cells as an indicator of splenic abnormality or risk of infection has been unclear. To assess changes in B cell sub-populations after splenectomy we studied patients recruited to a spleen registry (n = 591). A subset of 209 adult asplenic or hyposplenic subjects, and normal controls (n = 140) were tested for IgM memory B cells. We also determined a) changes in IgM memory B cells with time after splenectomy using the cross-sectional data from patients on the registry and b) the kinetics of changes in haematological markers associated with splenectomy(n = 45). Total B cells in splenectomy patients did not differ from controls, but memory B cells, IgM memory B cells and switched B cells were significantly (p<0.001) reduced. The reduction was similar for different indications for splenectomy. Changes of Asplenia in routine blood films including presence of Howell-Jolly bodies (HJB), occurred early (median 25 days) and splenectomy associated thrombocytosis and lymphocytosis peaked by 50 days. There was a more gradual decrease in IgM memory B cells reaching a stable level within 6 months after splenectomy. IgM memory B cells as proportion of B cells was the best discriminator between splenectomized patients and normal controls and at the optimal cut-off of 4.53, showed a true positive rate of 95% and false positive rate of 20%. In a survey of 152 registry patients stratified by IgM memory B cells around this cut-off there was no association with minor infections and no registry patients experienced OPSI during the study. Despite significant changes after splenectomy, conventional measures of IgM memory cells have limited clinical utility in this population.
Siegbert Rieg - One of the best experts on this subject based on the ideXlab platform.
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prevention of post splenectomy sepsis in patients with Asplenia a study protocol of a controlled trial
BMC Infectious Diseases, 2020Co-Authors: Marianne Bayrhuber, Natascha Anka, Johannes Camp, M Glattacker, Erik Farin, Siegbert RiegAbstract:Patients with Asplenia have a significantly increased lifelong risk of severe invasive infections, particular post-splenectomy sepsis (PSS). Clear preventive measures have been described in the literature, but previous studies found poor implementation of prevention recommendations. Aim of the study is to improve the adherence to guideline-based preventive measures and thereby reduce the incidence of PSS by a novel telephone-delivered intervention that involves both patients and their physicians. A prospective controlled, two-armed historical control group design is used to evaluate the new intervention compared to usual care. The intervention for patients includes both educational aspects and, building on the Health Action Process Approach (HAPA), intervention components that promote motivation and planning of preventive measures. For physicians the intervention is primarily information-based. The primary outcome, the adherence to preventative measures, is indicated by a study-specific ‘Preventing PSS-score’ (PrePSS-score), which is assessed at baseline and at 6-months follow-up. Secondary outcomes include, amongst others, patient self-efficacy and action-planning, Asplenia-specific health literacy, general self-management and Asplenia-specific self-management. In a process-evaluating part of the study interview-data on patients’ and physicians’ evaluation of the intervention will be gathered. This trial will provide evidence about the effectiveness of the novel prevention intervention for asplenic patients. If demonstrated beneficial, the intervention manual will be made publicly available to enable implementation in practice. The experience gained within this trial may also be valuable for prevention strategies in patients with other diseases. German Clinical Trials Register (DRKS): DRKS00015238; Trial registration date 07. December 2018.
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Prevention of post-splenectomy sepsis in patients with Asplenia - a study protocol of a controlled trial
BMC Infectious Diseases, 2020Co-Authors: Marianne Bayrhuber, Natascha Anka, Johannes Camp, M Glattacker, Erik Farin, Siegbert RiegAbstract:Background Patients with Asplenia have a significantly increased lifelong risk of severe invasive infections, particular post-splenectomy sepsis (PSS). Clear preventive measures have been described in the literature, but previous studies found poor implementation of prevention recommendations. Aim of the study is to improve the adherence to guideline-based preventive measures and thereby reduce the incidence of PSS by a novel telephone-delivered intervention that involves both patients and their physicians. Methods A prospective controlled, two-armed historical control group design is used to evaluate the new intervention compared to usual care. The intervention for patients includes both educational aspects and, building on the Health Action Process Approach (HAPA), intervention components that promote motivation and planning of preventive measures. For physicians the intervention is primarily information-based. The primary outcome, the adherence to preventative measures, is indicated by a study-specific ‘Preventing PSS-score’ (PrePSS-score), which is assessed at baseline and at 6-months follow-up. Secondary outcomes include, amongst others, patient self-efficacy and action-planning, Asplenia-specific health literacy, general self-management and Asplenia-specific self-management. In a process-evaluating part of the study interview-data on patients’ and physicians’ evaluation of the intervention will be gathered. Discussion This trial will provide evidence about the effectiveness of the novel prevention intervention for asplenic patients. If demonstrated beneficial, the intervention manual will be made publicly available to enable implementation in practice. The experience gained within this trial may also be valuable for prevention strategies in patients with other diseases. Trial registration German Clinical Trials Register (DRKS): DRKS00015238 ; Trial registration date 07. December 2018.
Allen C Cheng - One of the best experts on this subject based on the ideXlab platform.
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a registry for patients with Asplenia hyposplenism reduces the risk of infections with encapsulated organisms
Clinical Infectious Diseases, 2018Co-Authors: Alicia Arnott, Penelope Jones, Lucinda J Franklin, Denis Spelman, Karin Leder, Allen C ChengAbstract:Background Overwhelming post-splenectomy infection (OPSI) is a serious complication of Asplenia. Clinical guidelines recommend numerous measures to reduce the risk of OPSI, but awareness and adherence to preventative measures are generally poor. We aimed to determine whether a registry for asplenic/hyposplenic patients was associated with a reduction in the incidence of infection with encapsulated bacteria. Methods We performed a retrospective cohort study of asplenic/hyposplenic patients in the state of Victoria, Australia, who registered with Spleen Australia from 2003 through 2014. Spleen Australia provides education, clinical guidance, and annual vaccination reminders to registrants and their healthcare providers. We compared the incidence of infection with Streptococcus pneumoniae, Haemophilus influenzae type B (Hib), and Neisseria meningitidis before and after registration. Registry data were linked with Victorian notifiable disease data on invasive pneumococcal disease (IPD), invasive meningococcal disease (IMD), and Hib between 2000 and 2014. Results Twenty-seven cases of IPD and 1 of IMD occurred among 3221 registrants. No cases of Hib were reported. The rate of IPD/IMD was 150 per 100000 patient-years prior to registration and 36 per 100000 patient-years after registration; registration was associated with a 69% reduction in the risk of infection (incidence rate ratio, 0.31; 95% confidence interval, 0.12 to 0.83; P = .019). Based on the absolute reduction in incidence, we estimate that Spleen Australia prevents 5-6 invasive infections with encapsulated organisms annually among registrants. Conclusions Systematic, long-term approaches to post-splenectomy care can significantly reduce the risk of infection with encapsulated organisms among individuals with Asplenia/hyposplenism.
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overwhelming post splenectomy sepsis in patients with Asplenia and hyposplenia a retrospective cohort study
Epidemiology and Infection, 2017Co-Authors: Joash Wen Chen Chong, Penelope Jones, Denis Spelman, Karin Leder, Allen C ChengAbstract:Overwhelming post-splenectomy infection (OPSI) is a serious complication of Asplenia and is associated with encapsulated organisms, most commonly Streptococcus pneumoniae, but also Haemophilus influenzae and Neisseria meningitidis. We aimed to estimate the risk of infection in this patient group. We reviewed data collected by the Victorian Spleen Registry in Australia. On registration, all patients are asked about significant infections requiring admission to hospital for intravenous antibiotics; those requiring admission to ICU were defined as OPSI. In the 3274 asplenic patients registered 492 patients reported at least one episode of infection. There were 47 episodes of OPSI requiring intensive care (incidence rate 1·11/1000 patient-years). The risk of OPSI was highest in older patients, and there were no statistically significant differences in incidence by reason for splenectomy except for a higher rate in patients with medical hyposplenia. This study reinforces that post-splenectomy infection is a clinically significant but uncommon complication, and that fulminant infection requiring intensive care is a minority of all infections.
Koenraad Devriendt - One of the best experts on this subject based on the ideXlab platform.
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Agenesis of corpus callosum and anophthalmia in the Asplenia syndrome. A recognisable association
Annales de genetique, 1997Co-Authors: Koenraad Devriendt, Gunnar Naulaers, Gert Matthijs, K Van Houdt, Hugo Devlieger, Marc Gewillig, Jean-pierre FrynsAbstract:We present a newborn infant with the Asplenia syndrome and unique associated features of corpus callosum agenesis, anophthalmia and coloboma. Previous reports of eye abnormalities or corpus callosum agenesis in patients with Asplenia suggest that this may represent a distinct clinically recognisable entity of abnormal lateralisation.
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Asplenia syndrome and isolated total anomalous pulmonary venous connection in siblings.
European journal of pediatrics, 1994Co-Authors: Koenraad Devriendt, Marc Gewillig, A. Casaer, A. Van Cauter, F. De Zegher, Monique Dumoulin, Hugo DevliegerAbstract:We report on a family with Asplenia syndrome in one and total anomalous pulmonary venous connection (TAPVC) in the other sib. Both conditions are rare, may have a genetic cause and belong to a spectrum of laterality disorders. This suggests that both Asplenia syndrome and TAPVC in this family are the clinical expression of a single genetic disorder.
