%0 Report %D 2024 %T Annual report Belgian Cystic Fibrosis Registry 2020 %A Simeon Wanyama %A Géraldine Daneau %A Lieven Dupont %A Kim Van Hoorenbeeck %K Cystic Fibrosis %K mucoviscidose %I Sciensano %C Brussels, Belgium %P 120 %8 02/2024 %G eng %0 Report %D 2023 %T Annual Report Belgian Cystic Fibrosis Registry 2021 %A Simeon Wanyama %A Géraldine Daneau %A Lieven Dupont %A Kim Van Hoorenbeeck %K Cystic Fibrosis %K mucoviscidose %I Sciensano %C Brussels, Belgium %P 120 %8 11/2023 %G eng %M D/2023.14.440/72 %R 10.25608/vx5t-k083 %0 Conference Proceedings %D 2023 %T The Belgian Cystic Fibrosis Registry - Facts and highlights 2021 %A Simeon Wanyama %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %P 8 %8 12/2023 %0 Conference Proceedings %D 2023 %T Belgisch Mucoviscidose Register - Feiten en cijfers 2021 %A Simeon Wanyama %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %P 8 %8 12/2023 %0 Journal Article %J Belgian Journal of Paediatrics %D 2023 %T Clinical Evolution and Disease Burden in Belgian Cystic Fibrosis Patients: effect of Newborn Screening? %A Noelia Rodriguez Mier %A Vermeulen, François %A Géraldine Daneau %A Simeon Wanyama %A Proesmans, Marijke %X

Objectives: We compared the clinical evolution and disease burden of 3 groups of Belgian children with cystic fibrosis (CF) depending on the mode of diagnosis: meconium ileus (MI), newborn screening (NBS) and clinical diagnosis (non-NBS) at 1-, 2- and 6 years of age.Methods: We conducted a retrospective observational cohort study based on data collected from the Belgian CF Registry report of 2016 including patients diagnosed between January 2006 and December 2016.

Results: 306 CF patients were enrolled. Age at diagnosis was the lowest in MI patients (median 0.12 months, IQR 0.00-0.36 months) but lower in NBS patients (median 1.38 months, IQR 0.82-1.74 months) compared to non-NBS patients (8.01 months, IQR 2.47-37.58 months, p-value < 0.001). Height z-scores at 1 year of age were better in NBS patients (median = -0.13, IQR -0.99 – 0.50) compared to non-NBS patients (MI median = -0.56, IQR = -01.53 – 0.02, non-NBS median = -0.54, IQR = -1.02 – 0.12, p = 0.03377). Annual prevalence of bacterial infections was not different between the 3 patient groups at the predefined time points. Burden of therapy was lower for NBS during the first 2 years of life. Lung function at 6 years of age did not show significant differences between the 3 groups.

Conclusion: This study suggests a beneficial trend for NBS as primary method of CF detection when comparing age at diagnosis, clinical evolution and burden of therapy between NBS and clinically diagnosed CF patients. A standardized care program after CF-NBS is needed.

%B Belgian Journal of Paediatrics %V 23 %8 2023-01-18 %G eng %N 4 %& 292 %0 Journal Article %J Belgian Journal of Paediatrics %D 2023 %T Clinical Evolution and Disease Burden in Belgian Cystic Fibrosis Patients: effect of Newborn Screening? %A Noelia Rodriguez Mier %A Vermeulen, François %A Géraldine Daneau %A Simeon Wanyama %A Proesmans, Marijke %X

Objectives: We compared the clinical evolution and disease burden of 3 groups of Belgian children with cystic fibrosis (CF) depending on the mode of diagnosis: meconium ileus (MI), newborn screening (NBS) and clinical diagnosis (non-NBS) at 1-, 2- and 6 years of age.Methods: We conducted a retrospective observational cohort study based on data collected from the Belgian CF Registry report of 2016 including patients diagnosed between January 2006 and December 2016.

Results: 306 CF patients were enrolled. Age at diagnosis was the lowest in MI patients (median 0.12 months, IQR 0.00-0.36 months) but lower in NBS patients (median 1.38 months, IQR 0.82-1.74 months) compared to non-NBS patients (8.01 months, IQR 2.47-37.58 months, p-value < 0.001). Height z-scores at 1 year of age were better in NBS patients (median = -0.13, IQR -0.99 – 0.50) compared to non-NBS patients (MI median = -0.56, IQR = -01.53 – 0.02, non-NBS median = -0.54, IQR = -1.02 – 0.12, p = 0.03377). Annual prevalence of bacterial infections was not different between the 3 patient groups at the predefined time points. Burden of therapy was lower for NBS during the first 2 years of life. Lung function at 6 years of age did not show significant differences between the 3 groups.

Conclusion: This study suggests a beneficial trend for NBS as primary method of CF detection when comparing age at diagnosis, clinical evolution and burden of therapy between NBS and clinically diagnosed CF patients. A standardized care program after CF-NBS is needed.

%B Belgian Journal of Paediatrics %V 23 %8 2023-01-18 %G eng %N 4 %& 292 %0 Conference Proceedings %D 2023 %T Le Registre Belge de la Mucoviscidose - Faits et chiffres 2021 %A Simeon Wanyama %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %P 8 %8 12/2023 %0 Generic %D 2023 %T P142 Review of diagnostic labels for patients with unconfirmed cystic fibrosis diagnosis using registry data %A Simeon Wanyama %A Géraldine Daneau %A E. De Wachter %A L. Dupont %B Journal of Cystic Fibrosis %C Vienna, Austria %V 22 %8 Jan-06-2023 %G eng %R 10.1016/S1569-1993(23)00517-9 %0 Generic %D 2023 %T P146 COVID-19 pandemic in the Belgian cystic fibrosis patients compared with the international cystic fibrosis patients and the general Belgian population %A Géraldine Daneau %A Simeon Wanyama %A K. Van Hoorenbeeck %B Journal of Cystic Fibrosis %C Vienna, Austria %V 22 %8 Jan-06-2023 %G eng %R 10.1016/S1569-1993(23)00521-0 %0 Generic %D 2023 %T WS03.03 Clinical outcomes and long-term survival in cystic fibrosis (CF) lung transplant recipients in Belgium %A M. Thomas %A L. Dupont %A C. Knoop %A Simeon Wanyama %A P. Evrard %A B. Rondelet %A M. Vander Kuylen %A A. Van Muylem %A D. Van Raemdonck %B Journal of Cystic Fibrosis %C Vienna, Austria %V 22 %8 Jan-06-2023 %G eng %R 10.1016/S1569-1993(23)00201-1 %0 Generic %D 2022 %T ePS4.02 Impact of COVID-19 on the disease course in cystic fibrosis? A Registry-based study %A J. Vandekerckhove %A Simeon Wanyama %A Géraldine Daneau %A L. Peeters %A E. DeWachter %B Journal of Cystic Fibrosis %C Rotterdam, The Netherlands %V 21 %8 Jan-06-2022 %G eng %R 10.1016/S1569-1993(22)00310-1 %0 Report %D 2021 %T Annual Report Belgian Cystic Fibrosis Registry 2019 %A Simeon Wanyama %A Géraldine Daneau %A Dupont, Lieven %K annual report %K Cystic Fibrosis %K epidemiology %K mucoviscidose %I Sciensano %C Brussels, Belgium %P 104 %8 04/2021 %G eng %M D/2020/14.440/85 %R 10.25608/4gqx-wv11 %0 Report %D 2020 %T Annual Report Belgian Cystic Fibrosis Registry 2018 %A Simeon Wanyama %A Géraldine Daneau %A Dupont, Lieven %K annual report %K Cystic Fibrosis %K epidemiology %K mucoviscidose %X

 

SUMMARY

 

 

This report presents the data collected in 2018. It is our hope that the analysis of the registry data will provide readers with information on various aspects of CF and continue to provide an important tool for monitoring the patient’s quality of care and trends.  

 

Since its establishment in 1999, the Belgian CF Registry (BCFR) has grown steadily and had 1320 patients registered in 2018. This number excludes three patients whose diagnosis for CF was revoked and eight others without a confirmed diagnosis. We also miss 1-2% of patients who had not been seen between the introduction of the new GDPR-adapted Informed Consent Form and the data submission. There were 22 newly diagnosed patients in 2018, among them five adults, with a median age at diagnosis of 9.8 months and age range from birth to 70.6 years. All the newly diagnosed patients were genotyped;  18 had sweat chloride values > 60 mmol/L ; 2 had no sweat chloride value yet by December 2018.

 

Among the patients followed-up in 2018, 51.9% were male and 64.3% adults, and the median age was 23.6 years. At inception of the registry, 18 years ago, 39.0% were adults, and the median age was 14.9 years, showing changes in demographics over the years. 45.8% of the patients are homozygous for the F508del mutation and 38.0% are F508del heterozygous. The main reasons for diagnosis of CF are acute or recurrent respiratory problems (41.9%) and failure to thrive (24.9%). About 19.2% were diagnosed via neonatal screening even though Belgium had no national neonatal screening program at the time. Within the year, sixteen deaths were reported (nine of them among transplanted patients) with age at death ranging from 20.4 to 70.2 years. Sixteen patients benefitted from a lung transplant, and 14.3% of the patients in the registry are living with a transplant.

 

Among the adults, the proportion of underweight patients (BMI < 18.5 kg/m²), continues to decline from about 31.2% in 1998 to 16.7% in 2010 and 12.0% in 2018. This decline was noted also amongst the F508del homozygous patients; 32.8% in 2000, 21.9% in 2010, and 13.9% in 2018. Amongst the patients up to 20 years, the proportion with BMI below the tenth percentile has also been declining over the years. The results above suggest better nutritional and global management in the patients. The patient population continues to record an improvement in lung function expressed as the mean percentage of predicted FEV1. Among the F508del homozygous patients, 27.6% of the children and 3.5% of the adults had FEV1 ≥ 90.0% of predicted in 1998 compared to 49.5% and 8.4% in 2010 and 52.4% and 16.4% respectively among the children and adults in 2018.

 

The overall annual prevalence of Pseudomonas aeruginosa reported in 2018 was 37.9%. This is in line with the general decline observed from 42.4% in 2012 to 37.5% in 2016. There has also been a steady increase in the prevalence of Achromobacter xylosoxidans from 5.9% in 2009, stabilizing at prevalence levels of about 10.0% since 2012. The prevalence of the Burkholderia cepacia complex on the other hand has decreased to 3.0% in 2018, after a stable period at about 3.5% over the years since 2014.

 

Thanks to improved disease management and novel treatments, the life expectancy and the quality of life of patients with CF has improved significantly when compared to CF cohorts a decade or two ago. Cystic Fibrosis is no longer a paediatric disease, however this progress is also accompanied by various challenges, expectations and disease-related complications. In 2018, CF-related diabetes had a prevalence of 4.3% and 29.3% (non-transplanted) in children and adults respectively. Other reported complications include early osteoporosis and CF related arthritis/arthropathy. These require specialized care for the adult CF patients.

%I Sciensano %C Brussels, Belgium %P 104 %8 08/2020 %G eng %M D/2020/14.440/40 %R 10.25608/xgz4-rh13 %0 Conference Proceedings %D 2020 %T The Belgian Cystic Fibrosis Registry - Facts and highlights 2018 %A Simeon Wanyama %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %8 08/2020 %0 Conference Proceedings %D 2020 %T Belgisch Mucoviscidose Register - Feiten en cijfers 2018 %A Simeon Wanyama %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %8 08/2020 %0 Thesis %D 2020 %T Clinical Evolution and Disease Burden of Cystic Fibrosis Patients after Newborn Screening and Clinical Diagnosis %A Noelia Rodriguez Mier %A Simeon Wanyama %A Géraldine Daneau %A Proesmans, Marijke %K Cystic Fibrosis %K mucoviscidose %K Neonatal Screening %I KU Leuven %C Leuven, Belgium %8 02/2020 %G eng %0 Conference Proceedings %D 2020 %T Le Registre Belge de la Mucoviscidose - Faits et chiffres 2018 %A Simeon Wanyama %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %8 08/2020 %0 Report %D 2019 %T Annual Report Belgian Cystic Fibrosis Registry 2017 %A Simeon Wanyama %A Murielle Thomas %A Géraldine Daneau %K annual report %K Cystic Fibrosis %K epidemiology %K mucoviscidose %X This report presents the data collected in 2017. It is our hope that the analysis of the registry data will provide readers with information on various aspects of CF and continue to provide an important tool for monitoring the patient’s quality of care and trends. Since its establishment in 1998, the Belgian CF Registry (BCFR) has grown steadily and had 1319 patients registered in 2017. This number excludes four patients whose diagnosis for CF was revoked and fourteen without a confirmed diagnosis. There were 38 newly diagnosed patients in 2017, among them two adults, with a median age at diagnosis of 5.4 months with a range from birth to 60.4 years. All the newly diagnosed patients were genotyped; 34 had sweat chloride values > 60 mmol/L. Among the patients followed-up in 2017, 51.8% were male and 62.2% adults, and the median age was 22.8 years. Interestingly, at the start of the registry, 18 years ago, 39.0% were adults, and the median age was 14.9 years. 46.4% of the patients are homozygous for the F508del mutation and 39.1% are F508del heterozygous. The main reasons for diagnosis of CF are acute or recurrent respiratory problems (42.2%) and failure to thrive (24.4%). About 18.7% were diagnosed via neonatal screening even though Belgium had no national neonatal screening program at the time. Within the year, six deaths were reported (two of them in transplanted patients) with age at death ranging from 11.8 to 70.4 years. Ten patients benefitted from a lung transplant, and 13.9% of the patients in the registry are living with a transplant. Among the adults, the proportion of underweight patients continues to decline from about 31.2% in 1998 to 16.7% in 2010 and 10.8% in 2017 (BMI < 18.5 kg/m²); this decline was noted also amongst the F508del homozygous patients; 32.8% in 2000, 21.9% in 2010, and 15.8% in 2017. Amongst the patients up to 20 years, the proportion with BMI below the tenth percentile has also been declining over the years. The above suggests better nutritional management in the patients. The patient population continues to record an improvement in lung function expressed as the mean percentage of predicted FEV1. Among the F508del homozygous patients, 38.0% of the children and 5.1% of the adults had FEV1 ≥ 90.0% of predicted in 1998 compared to 52.9% and 7.0% in 2010 and 54.8% and 11.3% respectively among the children and adults in 2017. The overall annual prevalence of Pseudomonas aeruginosa reported in 2017 was 39.1%. This is the first time the prevalence has increased since the decline observed from 42.4% in 2012 to 37.5% in 2016. There has also been a steady increase in the prevalence of Achromobacter xylosoxidans from 5.9% in 2009, stabilizing at prevalence levels above 10.0% since 2012, but reaching 11.4% in 2017. The prevalence of the Burkholderia cepacia complex on the other hand has decreased to 2.9%, after a stable period at 3.5% over the years since 2014. Thanks to improved disease management and novel treatments, the life expectancy and the quality of life of patients with CF has improved significantly when compared to CF cohorts a decade or two ago. The proportion of adult CF patients aged 18 years and above increases each year. But this progress is also accompanied by various challenges, expectations and disease-related complications. In 2017, CF-related diabetes had a prevalence of 18.7% and 66.1% in non-transplanted and in transplanted adults respectively. Other complications reported include early osteoporosis and CF related arthritis/arthropathy. These require specialized care for the adult CF patients. %I Sciensano %C Brussels, Belgium %P 100 %8 11/2019 %G eng %M D/2019/14.440/16 %R 10.25608/f9pc-hd24 %0 Conference Proceedings %D 2019 %T The Belgian Cystic Fibrosis Registry - Facts and highlights 2017 %A Simeon Wanyama %A Murielle Thomas %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %8 11/2019 %0 Conference Proceedings %D 2019 %T Belgisch Mucoviscidose Register - Feiten en cijfers 2017 %A Simeon Wanyama %A Murielle Thomas %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %8 11/2019 %0 Conference Proceedings %D 2019 %T Le Registre Belge de la Mucoviscidose - Faits et chiffres 2017 %A Simeon Wanyama %A Murielle Thomas %A Géraldine Daneau %K Cystic Fibrosis %K mucoviscidose %8 11/2019 %0 Report %D 2018 %T Annual report Belgian Cystic Fibrosis Registry 2016 %A Simeon Wanyama %A Muriel Thomas %K Belgian cystic fibrosis registry %K Belgium %K Cystic Fibrosis %X

This report presents the data collected in 2016. It is our hope that the analysis of the registry data will provide readers with information on various aspects of CF and continue to provide an important tool for monitoring the patient’s quality of care and trends. In this edition, a brief analysis of anthropometry, spirometry, common complications and therapy in transplant patients has been added.

Since its establishment in 1998, the Belgium CF Registry (BCFR) has grown steadily and had 1275 patients registered in 2016. This number excludes five patients whose diagnosis for CF was revoked and fifteen without a confirmed diagnosis. There were 23 newly diagnosed patients in 2016, among them three adults, with a median age at diagnosis of 3.8 months with a range from birth to 52.3 years. All the newly diagnosed patients were genotyped; while 22 had sweat chloride values > 60 mmol/L.

Among the patients in follow-up in 2016, 52.0% were male and 61.2% adults with a median age of 22.5 years. This can be compared to the start of the registry 17 years ago when 39.0% were adults with a median age of 14.9 years. 46.7% of the patients are homozygous for the F508del mutation while 37.0% are F508del heterozygous. The main reasons for diagnosis of CF are acute or recurrent respiratory problems (42.1%) and failure to thrive (24.4%). About 18.0% were diagnosed via neonatal screening even though Belgium has no national neonatal screening program so far. Within the year, eight deaths were reported (four of them in transplanted patients) with age at death ranging from 20.5 to 44.8 years while 17 patients benefitted from a lung transplant. About 14.0% of the patients in the registry are living with a transplant.

Among the adults, the proportion of patients with BMI < 18.0 kg/m² continues to decline from about 36.3% in 1998 to 17.4% in 2010 and 11.7% in 2016; this decline was noted also amongst the F508del homozygous patients. Amongst the patients up to 20 years, the proportion with BMI below the tenth percentile has also been declining over the years. The above suggests better nutritional management in the patients. The patient population continues to record an improvement in lung function expressed as the mean percentage of predicted FEV1. Among the F508del homozygous patients, 38.0% of the children and 5.1% of the adults had FEV1 ≥ 90.0% of predicted in 1998 compared to 52.9% and 7.0% in 2010 and 53.7% and 13.6% respectively among the children and adults in 2016.

The overall annual prevalence of Pseudomonas aeruginosa reported in 2016 was 37.5% and has been declining compared to a prevalence of 42.4% in 2012. This prevalence has been below 40.0% since 2015. The prevalence of the Burkholderia cepacia complex on the other hand had remained stable over the years since 2014 at about 3.5% There has also been a steady 14 Summ ar y increase in the prevalence of Achromobacter xylosoxidans from 5.9% in 2009 stabilizing at prevalence levels above 10.0% since 2012.

Thanks to improved disease management practises and novel treatments, the life expectancy and the quality of life of patients with CF has improved significantly when compared to CF cohorts a decade or two ago. The proportion of adult CF patients aged 18 years and above increases each year. But this progress is also accompanied by different challenges, expectations and disease related complications. In 2016, CF related diabetes had a prevalence of 24.5% and 53.1% in non- transplanted and transplanted adults respectively. Other complications reported include early osteoporosis and CF related arthritis/arthropathy. These require specialized care for the adult CF patient.

%P 100 %8 03/2018 %G eng %0 Conference Proceedings %D 2018 %T The Belgian Cystic Fibrosis Registry - Facts and highlights 2016 %A Simeon Wanyama %A Muriel Thomas %K Cystic Fibrosis %K highlights %P 8 %8 2018 %0 Conference Proceedings %D 2018 %T Belgisch mucoviscidose register - Feiten en cijfers 2016 %A Simeon Wanyama %A Muriel Thomas %P 8 %8 2018 %0 Journal Article %J J Cyst Fibros %D 2018 %T The effect of enteral tube feeding in cystic fibrosis: A registry based study. %A Denis Libeert %A Dimitri Declercq %A Simeon Wanyama %A Muriel Thomas %A Sabine Van Daele %A Frans De Baets %A Stephanie Van Biervliet %K Cystic Fibrosis %K Gastrostomy %K Malnutrition %K Pulmonary function %K Tube feeding %X

BACKGROUND: Long-term effect of enteral tube feeding (ETF) in cystic fibrosis (CF) remains equivocal.

METHODS: A Belgian CF registry based, retrospective, longitudinal study, evaluated the pre- and post- ETF (n = 113) clinical evolution and compared each patient with 2 age, gender, pancreatic status and genotype class-matched controls.

RESULTS: At baseline ETF had a worse BMI z-score (p < 0.0001) and FEV1% (p < 0.0001) compared to controls. Patients eventually receiving ETF, had already a significant worse nutritional status and pulmonary function at first entry in the registry. Both parameters displayed a significant decline before ETF-introduction. ETF had more hospitalization and intravenous antibiotic (IVAB) treatment days (p < 0.0001). After ETF introduction hospitalizations and IVAB decreased significantly. After ETF-introduction BMI z-score recuperated towards the original curve before the decline, but remained below the controls. Starting ETF had no effect on rate of height gain in children. The pre-index FEV1 decline (-1.52%/year (p = 0.002)) stabilized to +0.39%/year afterwards. Controls displayed decline of -0.48%/year (p < 0.0001).

CONCLUSION: ETF introduction improved BMI z-score and stabilized FEV1, associated with less hospitalizations and IVAB treatments. Higher mortality and transplantation in the ETF cases, leading to drop-outs, made determination of the effect size difficult.

%B J Cyst Fibros %V 17 %8 2018 Mar %G eng %N 2 %R 10.1016/j.jcf.2018.01.004 %0 Generic %D 2018 %T EPS5.06 Demography and clinical outcomes in cystic fibrosis lung transplant recipients in Belgium %A Muriel Thomas %A Simeon Wanyama %A L Dupont %A I. Etienne %A P. Evrard %A B. Rondelet %A Y. Sokolow %A D. Van Raemdonck %K Belgium %K Cystic Fibrosis %K lung transplant %B Journal of Cystic Fibrosis %V 17 %8 Jan-06-2018 %G eng %R 10.1016/S1569-1993(18)30266-2 %0 Conference Proceedings %D 2018 %T Le registre belge de la mucoviscidose - Faits et chiffres 2016 %A Simeon Wanyama %A Muriel Thomas %P 8 %8 2018 %0 Generic %D 2018 %T Quantifying activity changes of neuromuscular patients using the ACTIVLIM questionnaire: a 5-years longitudinal study %A Corinne Bleyenheuft %A N. Goemans %A Simeon Wanyama %A P. Van Damme %A J. De Bleecker %A R. Van Coster %A P. De Jonghe %A D. Beysen %A P. Van den Bergh %A S. Paquay %A L. Servais %A A. Maertens de Noordhout %A J. Haan %A L. De Meirleir %A G. Remiche %A N. Deconinck %A C. Arnould %B 23rd International Annual Congress of the World Muscle Society (WMS) %G eng %R 10.1016/j.nmd.2018.06.332 %0 Generic %D 2018 %T Quantifying the changes in activity level of neuromuscular patients using the ACTIVLIM questionnaire: A 5-years study %A Corinne Bleyenheuft %A Simeon Wanyama %A P. Van Damme %A N. Goemans %A J. De Bleecker %A R. Van Coster %A P. De Jonghe %A D. Beysen %A P. Van den Bergh %A S. Paquay %A L. Servais %A A. Maertens de Noordhout %A J. Haan %A L. De Meirleir %A G. Remiche %A N. Deconinck %A C. Arnould %A BNMDR study group %K Activity %K assessment %K Neuromuscular Diseases %B 12th ISPRM World Congress %G eng %R 10.1016/j.rehab.2018.05.1260 %0 Journal Article %J Pediatr Allergy Immunol %D 2018 %T Risk factors and impact of allergic bronchopulmonary aspergillosis in Pseudomonas aeruginosa-negative CF patients. %A Frans De Baets %A Linde De Keyzer %A Sabine Van Daele %A Petra Schelstraete %A Stephanie Van Biervliet %A Eva Van Braeckel %A Muriel Thomas %A Simeon Wanyama %K allergic bronchopulmonary aspergillosis %K Aspergillus colonization %K Aspergillus fumigatus %K Aspergillus sensitization %K Cystic Fibrosis %X

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a major complication in cystic fibrosis (CF) patients. Risk factors for ABPA and clinical deterioration in CF patients, negative for Pseudomonas aeruginosa (Pa), were explored.

METHODS: We performed a retrospective case-control study in 73 Pa-negative patients. Each patient was matched with 2 controls for age, gender, pancreas sufficiency, DeltaF508 mutation (homozygous or heterozygous), and Pa colonization.

RESULTS: Median FEV at the year of diagnosis (index year) was significantly lower in patients with ABPA. The median of cumulative values of FEV and FVC before the index year was not significantly different. After the index year, the median of cumulative data for FEV and FVC was significantly lower; there were significantly more hospitalization days and more IV antibiotic days compared to controls. Comparing pre- and post-index year data in patients with ABPA, significantly more hospitalization days and more IV antibiotic days were observed after the index year. During the period preceding the index year, significantly more ABPA patients were treated with rhDNase and inhaled corticosteroids.

CONCLUSIONS: Bronchial damage cannot be considered as a facilitating factor for ABPA. ABPA causes a significant increase in bronchial damage. In patients with ABPA, further bronchial damage can be controlled by an increase in hospitalization days and use of IV antibiotics. rhDNase and inhaled corticosteroids were associated with the development of ABPA.

%B Pediatr Allergy Immunol %8 2018 Jul 07 %G eng %R 10.1111/pai.12953 %0 Report %D 2017 %T Annual report Belgian Cystic Fibrosis Registry 2014 %A Simeon Wanyama %A Muriel Thomas %A Malfroot, Anne %P 96 %8 05/2017 %G eng %M D/2017/2505/04 %0 Report %D 2017 %T Annual report Belgian Cystic Fibrosis Registry 2015 %A Simeon Wanyama %A Muriel Thomas %A Malfroot, Anne %P 103 %8 12/2017 %G eng %M D/2017/2505/31 %0 Journal Article %J Eur Respir J %D 2017 %T Does newborn screening influence the young cystic fibrosis cohort included in national registries? %A De Boeck, Kris %A Munck, Anne %A de Monestrol, Isabelle %A Gulmans, Vincent %A Lemonnier, Lydie %A Middleton, Peter G %A Simeon Wanyama %A Muriel Thomas %B Eur Respir J %V 49 %8 2017 Jan %G eng %N 1 %1 http://www.ncbi.nlm.nih.gov/pubmed/28077474?dopt=Abstract %R 10.1183/13993003.00686-2016 %0 Journal Article %J Orphanet J Rare Dis %D 2017 %T What can the CF registry tell us about rare CFTR-mutations? A Belgian study. %A De Wachter, E %A Muriel Thomas %A Simeon Wanyama %A Seneca, S %A Malfroot, A %X

BACKGROUND: CFTR2 provides clinical and functional information of the most common CFTR-mutations. Rare mutations (RMs) occur in only a few patients with limited reported clinical data. Their role in CF-disease liability is hardly documented.

METHODS: Belgian CF-Registry 2013 data were analyzed to identify CF with at least 1 RM (CF+RM). Clinical data and sweat chloride of CF+RM were compared to CF-controls, carrying 2 class 1 to 3 mutations (CFclassic). Disease severity was compared between both groups. To avoid bias in the comparison, transplanted patients were excluded from each group.

RESULTS: Seventy-seven CF+RM were identified (77/1183 = 6.5%). Sixty-four different RM were detected, of which 21 had not been previously reported. All RMs, corresponding to HGVS (Human Genome Variation Society) nomenclature, were listed in supplementary data. Seven transplanted CF+RM were excluded for further analysis. CF+RM had higher age at diagnosis [median (IQR)] [3.7 y (0.3-18.3) vs. 0.3y (0.1-2,0) (p < 0.0001)], lower sweat chloride [96 mmol/L (64-107) vs. 104 mmol/L (97-115) (p < 0.0001)], higher FEV1%pred [77%pred (58-96) vs. 68%pred (48-86) (p = 0.017)], were less frequently pancreatic insufficient [56% vs. 98% (p < 0.0001)], Pseudomonas aeruginosa colonized [24% vs. 44% (p = 0.0093)] and needed fewer IV antibiotics [36% vs. 51% (p = 0.041)] than CFclassic. However, a wide spectrum of disease severity was seen amongst CF+RM.

CONCLUSIONS: CF-patients with a RM cover 6.5% of the Belgian CF-population. Rare mutations can be found in severely ill patients, but more often in late diagnosed, pancreatic sufficient patients.

%B Orphanet J Rare Dis %V 12 %P 142 %8 2017 Aug 22 %G eng %N 1 %1 http://www.ncbi.nlm.nih.gov/pubmed/28830496?dopt=Abstract %R 10.1186/s13023-017-0694-1 %0 Journal Article %J BMC Public Health %D 2016 %T General practice patients treated for substance use problems: a cross-national observational study in Belgium. %A Nicole Boffin %A Jérôme Antoine %A S Moreels %A Simeon Wanyama %A Karin De Ridder %A Peremans, Lieve %A Vanmeerbeek, Marc %A Viviane Van Casteren %K Adult %K Belgium %K Female %K General practice %K general practitioners %K Humans %K Male %K Methadone %K middle aged %K Prescription Drugs %K Substance-Related Disorders %X

BACKGROUND: General Practitioners (GPs) are well placed to care for patients with (chronic) substance use problems. This pilot was carried out to study the feasibility and usefulness of a continuous surveillance of substance use problems among general practice patients. The objectives were (i) to describe variables with missing values exceeding 1% and whether patients were reported without substance-related problems; (ii) the profile and the magnitude of the patient population that is treated for substance use problems.

METHODS: Observational study by the Belgian Network of Sentinel General Practices (SGP) in 2013. Baseline (at the first encounter) and 7-month follow-up data were reported of all patients treated for substance use problems. Two main measurements were type of substance use and patient status at follow-up. Multiple logistic regression analysis was used to examine patient status at follow-up.

RESULTS: Of 479 patients, 47.2% had problems with alcohol alone, 20.3% with prescription drugs, 16.7% with illicit drugs other than heroin or methadone and 15.9% with heroin or methadone. Problems with alcohol alone were more prevalent in Flanders (53.0%; 95% confidence interval (CI) 46.8-59.1%) than in Wallonia-Brussels (39.8%; 95% CI 33.1-46.8%), while problems with heroin or methadone were more prevalent in Wallonia-Brussels (27.0%; 95% CI 21.1-33.5%) than in Flanders (7.1%; 95% CI 4.3-10.9%). At follow-up, 32.8% of the patients had dropped out, 29.0% had discontinued GP treatment and 38.2% had continued GP treatment. Overall, 32.4% of 479 patients had continued GP treatment for substance use problems during the study period. In Wallonia-Brussels, this proportion was higher (42.7%; 95% CI 35.9-49.6%) than in Flanders (24.3%; 95% CI 19.2-29.8%).

CONCLUSIONS: A continuous surveillance of the general practice population treated for substance use problems seems to be feasible and useful. The latter is suggested by the specific profile and the relative magnitude of the population. Inter-regional health system differences should be taken into account to estimate the epidemiology of substance use problems among general practice patients.

%B BMC Public Health %V 16 %P 1235 %8 2016 Dec 08 %G eng %N 1 %1 http://www.ncbi.nlm.nih.gov/pubmed/27927240?dopt=Abstract %R 10.1186/s12889-016-3885-0 %0 Report %D 2015 %T Annual Report Belgian Cystic Fibrosis Registry 2013. %A Simeon Wanyama %A Muriel Thomas %A Malfroot,A. %K annual report %K Belgian %K Belgium %K Cystic Fibrosis %K data %K Fibrosis %K REGISTRIES %K Registry %K report %I WIV-ISP %C Brussels/Belgium %P 84 %8 30/5/2015 %@ D/2015/2505/30 %G eng %1

1368

%& 1 %0 Report %D 2015 %T Jaarlijks Rapport Belgisch Mucoviscidose Register 2013. %A Simeon Wanyama %A Muriel Thomas %A Malfroot,A. %K Belgium %K Cystic Fibrosis %K data %K de %K Fibrosis %K mucoviscidose %K rapport %K Register %K REGISTRIES %K Registry %K report %X

In dit rapport worden de gegevens voorgesteld, verzameld in 2013. We hopen dat dit rapport de lezers zal verder toelichten over de aspecten van mucoviscidose in het algemeen en over de zorg die ter beschikking staat voor de personen met mucoviscidose in België in het bijzonder.

Sinds zijn oprichting is het aantal patiënten in het Belgisch Mucoviscidose Register gestaag gegroeid en in 2013 waren er 1186 patiënten in het BMRRBM geregistreerd. Dit aantal sluit 7 personen uit bij wie de diagnose van mucoviscidose werd weerlegd in 2013 en 7 bij wie de diagnose niet bevestigd was. Er waren 28 nieuw gediagnosticeerde patiënten in 2013, waaronder 2 volwassenen; de mediane leeftijd op het ogenblik van de diagnose voor de nieuw gediagnosticeerde patiënten was 8.2 maanden, met een spreiding van 0.0 – 25.7 jaar. De meeste van de nieuwe patiënten kregen ook een genetisch onderzoek met opsporing van de mutaties.

Onder de patiënten geregistreerd en gevolgd in 2013 behoorden 52.0% tot het mannelijk geslacht en waren 57.0% volwassenen, mediane leeftijd van alle patiënten was 20.7 jaar. Bij de start van het register 15 jaar geleden, waren slechts 39.0% volwassenen en was de mediane leeftijd 14.9 jaar. In 2013, waren 45.4% van de patiënten homozygoot voor de F508del mutatie, terwijl 40.4% heterozygoot waren voor F508del. De belangrijkste aanleidingen voor de diagnose van mucoviscidose bleven acute of recidiverende respiratoire problemen (43.6%) en slechte gewichts- en groeicurven (24.9%). In ongeveer 17.0% werd de diagnose gesteld door neonatale screening zelfs zonder aanwezigheid van een nationaal neonataal screening programma in België. Tijdens het jaar 2013 waren er 5 overlijdens (waarvan 2 bij getransplanteerde patiënten), leeftijd van overlijden varieerde van 17.1 – 30.1 jaar; 5 patiënten ondergingen een longtransplantatie.

Bij de volwassenen, bleef het aandeel patiënten met een BMI < 18.0 kg/m² verder dalen van ongeveer 36.3% in 1998 tot 17.4% in 2010 en 14.3% in 2013; deze daling werd zelfs vastgesteld bij de F508del homozygote patiënten. Ook bij de patiënten onder de leeftijd van 20 jaar, was het aandeel met een BMI onder percentiel 10 blijven dalen over de jaren heen. Deze cijfers illustreren een betere nutritionele aanpak van de patiënten. De patiëntenpopulatie bleef ook een continue verbetering vertonen van de longfunctie parameters, uitgedrukt in het gemiddelde percentage van de voorspelde éénseconde waarde (ESW). Onder de F508del homozygote patiënten, hadden in 1998, 38.0% van de kinderen en 5.1% van de volwassenen, in 2010 respectievelijk 52.9% en 7.0% en in 2013 respectievelijk zelfs 57.4% en 8.7% een ESW ≥ 90.0% van de voorspelde waarde.

De jaarlijkse prevalentie van Pseudomonas aeruginosa gerapporteerd in 2013 was 42.2% en was stabiel vergeleken met de prevalentie van 41.8% gerapporteerd in 2012. De prevalentie van Burkholderia cepacia complex bleef lager dan 3.0% gedurende jaren tot in 2010. In 2011 echter was de prevalentie gestegen tot 3.6%, (statistisch niet significant), en in 2012 en 2013 werd verder een stijgende trend van respectievelijk 4.0% en 4.5% vastgesteld; deze beide cijfers waren wel statistisch significant ten opzichte van de prevalentie van 2.4% in 2010. Er was tevens een toename in de prevalentie van Achromobacter xylosoxidans, een opkomende ziektekiem, van 5.9% in 2009 tot 10.5% in 2013.

Dankzij verbetering van de behandeling zijn de levensverwachting en de levenskwaliteit van de patiënten met mucoviscidose gestegen. Het aandeel patiënten met CF ouder dan 18 jaar neemt elk jaar toe. Maar deze vooruitgang gaat ook gepaard met een toename van complicaties bij volwassenen. CF gerelateerde diabetes had in 2013 een prevalentie van 26.6% bij niet getransplanteerde volwassenen. Andere complicaties waren vroege osteoporose, CF- gerelateerde artritis/artropathie ... Deze evolutie vereist een specifieke zorg voor volwassene patiënten.

%I WIV-ISP %C Brussels/Belgium %P 84 %8 30/5/2015 %@ D/2015/2505/32 %G eng %1

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%& 1 %0 Report %D 2015 %T Rapport Annuel Registre Belge de la Mucoviscidose 2013. %A Simeon Wanyama %A Muriel Thomas %A Malfroot,A. %K Belge %K Belgium %K Cystic Fibrosis %K data %K de %K Fibrosis %K mucoviscidose %K rapport %K rapport annuel %K registre %K REGISTRIES %K Registry %K report %I WIV-ISP %C Brussels/Belgium %P 84 %8 30/5/2015 %@ D/2015/2505/31 %G eng %1

1369

%& 1 %0 Journal Article %J Pediatr Pulmonol %D 2015 %T Treatment burden in patients with at least one class IV or V CFTR mutation %A Dewulf, Jonas %A Vermeulen, François %A Simeon Wanyama %A Muriel Thomas %A Proesmans, Marijke %A Dupont, Lieven %A De Boeck, Kris %K ADOLESCENT %K Ambulatory Care Facilities %K Anti-Bacterial Agents %K Child %K Child, Preschool %K Cohort Studies %K Cystic Fibrosis %K Cystic Fibrosis Transmembrane Conductance Regulator %K Drug Utilization %K Female %K Genotype %K Hospitalization %K Humans %K Infant %K Infant, Newborn %K Male %K Mutation %K Organ Transplantation %K REGISTRIES %K Respiratory Therapy %K Retrospective Studies %K Severity of Illness Index %K Young adult %X

CFTR mutations are grouped according to disease-causing mechanism. Several studies demonstrated that patients having at least one mutation of class IV/V, present with a milder phenotype, but little is known about their relative treatment burden. We compared treatment burden between patients with two class I, II, or III mutations and patients with at least one mutation of class IV/V in the 2010 database of the Belgian CF Registry. We calculated a "Treatment Burden Index" (TBI) by assigning long term therapies to categories low, medium and high intensity, for differential weighing in the total score. There were 779 patients with two known class I/II/III mutations and 94 patients with at least one class IV/V mutation. Compared to class I/II/III, class IV/V patients had a lower median number of clinic visits (4 vs. 5; P < 0.001), a lower risk of hospitalization (24.7% vs. 50.8%; P < 0.001) and intravenous antibiotic treatment (23.5% vs. 46.0%; P < 0.001) and a lower median TBI (6 vs. 9; P < 0.001). These differences remained significant when only class IV/V patients with pancreatic insufficiency (n = 31) were considered. This study clearly demonstrates the significantly lower treatment burden in patients with CF and at least one class IV/V mutation compared to patients with two class I/II/III mutations and contributes to providing better individual counseling at time of diagnosis.

%B Pediatr Pulmonol %V 50 %P 1230-6 %8 2015 Dec %G eng %N 12 %1 http://www.ncbi.nlm.nih.gov/pubmed/26540286?dopt=Abstract %R 10.1002/ppul.23313 %0 Report %D 2014 %T Annual report Belgian Cystic Fibrosis Registry 2012 %A Simeon Wanyama %A Muriel Thomas %P 76 %8 10/2014 %G eng %M D/2014/2505/57 %0 Report %D 2014 %T The Belgian Cystic Fibrosis Registry Summary Report 2011 %A Muriel Thomas %A Simeon Wanyama %A François Vermeulen %P 44 %8 2014 %G eng %M D/2014/2505/02 %0 Report %D 2014 %T Het Belgisch Mucoviscidose Register Beknopt Verslag 2011 %A Muriel Thomas %A Simeon Wanyama %A François Vermeulen %P 44 %8 2014 %G eng %M D/2014/2505/34 %0 Report %D 2014 %T Registre Belge de la Mucoviscidose Synthèse du Rapport 2011 %A Muriel Thomas %A Simeon Wanyama %A François Vermeulen %P 44 %8 2014 %G eng %M D/2014/2505/33 %0 Report %D 2013 %T Annual data report 2011 Cystic Fibrosis, Belgium. %A Simeon Wanyama %A Muriel Thomas %K Belgium %K Cystic Fibrosis %K data %K Fibrosis %K report %I WIV-ISP %C Brussels %P ? %8 0/0/2013 %@ D/2013/2505/43 %G eng %1 1360 %0 Generic %D 2013 %T Health related quality of life assessment amongst people with cystic fibrosis in Belgium %A Simeon Wanyama %A Sevens,C. %A Malfroot,A. %A Knoop,C. %A A.M. Depoorter %K assessment %K Belgium %K conference %K Cystic Fibrosis %K European %K Fibrosis %K health %K Life %K People %K public %K public health %K Public-health %K Quality %K Quality of Life %K use %B 6th European Public Health Conference %8 0/6/2013 %G eng %N EUPHA %1 1358 %2 13-16 November 2013 %0 Generic %D 2013 %T Quality of life assessment in cystic fibrosis - Use of the Rasch latent trait model. %A Simeon Wanyama %A Sevens,C. %A Malfroot,A. %A Knoop,C. %K assessment %K conference %K Cystic Fibrosis %K European %K Fibrosis %K Life %K Quality %K use %B 36th European Cystic Fibrosis Conference %I NA %C NA %8 0/6/2013 %G eng %N ECFC %1 1357 %2 12-15 June 2013 %0 Report %D 2012 %T The Belgian Cystic Fibrosis Registry Summary Report 2009 %A Muriel Thomas %A Simeon Wanyama %A Herwig Jansens %A François Vermeulen %P 44 %8 2012 %G eng %M D/2012/2505/22 %0 Report %D 2012 %T The Belgian Cystic Fibrosis Registry Summary Report 2010 %A Muriel Thomas %A Simeon Wanyama %A François Vermeulen %P 40 %8 2012 %G eng %M D/2012/2505/45 %0 Report %D 2012 %T The Belgian cystic fibrosis registry: summary report 2010 %A Muriel Thomas %A Simeon Wanyama %A Vermeulen,F. %E Johan Peeters %K 2010 %K Belgian %K Cystic Fibrosis %K Fibrosis %K REGISTRIES %K Registry %K report %K summary %X Not available %I WIV-ISP %C Brussels %P 40 %8 0/0/2012 %@ D/2012/2505/45 %G eng %1 1326 %& 1 %0 Report %D 2012 %T Het Belgisch Mucoviscidose Register Beknopt Verslag 2010 %A Muriel Thomas %A Simeon Wanyama %A François Vermeulen %P 10 %8 2012 %G eng %M D/2012/2505/68 %0 Generic %D 2012 %T Increased proportion of CF patients with normal FEV1 over an 11-years nation-wide study ? Have characteristics changed ? %A De Wachter,E. %A De Schutter,I. %A Muriel Thomas %A Simeon Wanyama %A Haentjes,P. %A Malfroot,A. %K an %K conference %K Cystic Fibrosis %K European %K Fibrosis %K Patient %K patients %K study %X

Not available

%B European Cystic Fibrosis Conference %I NA %C NA %8 0/0/2012 %G eng %N ECFS %1 1328 %2 June 2012 %0 Generic %D 2012 %T Problématique du dépistage systématique de la mucoviscidose. Données du registre Belge de la Mucoviscidose. %A Muriel Thomas %A Simeon Wanyama %A Jansen,H. %A Viviane Van Casteren %K Belge %K de %K mucoviscidose %X

Not available

%B Séminaire de concertation "problématique du dépistage néonatal systématique de la mucoviscidose" %I NA %C NA %8 0/3/2012 %G eng %N Fédération Wallonie-Bruxelles %1 35609 %2 Mars 2012 %0 Report %D 2012 %T Registre Belge de la Mucoviscidose Synthèse du Rapport 2010 %A Muriel Thomas %A Simeon Wanyama %A François Vermeulen %P 40 %8 2012 %G eng %M Rapport D/2012/2505/56 %0 Generic %D 2012 %T The relationship between socioeconomic status and clinical outcomes among cystic fibrosis patients in Belgium %A Simeon Wanyama %E Health Economics, Management and Policy %K Belgium %K Clinical %K conference %K Cystic Fibrosis %K ECONOMIC %K economics %K European %K Fibrosis %K health %K Health economic %K International %K IS %K management %K ON %K outcome %K outcomes %K Patient %K patients %K POLICIES %K POLICY %K REGISTRIES %K Registry %K Socioeconomic %K Socioeconomic status %K Socioeconomic-status %K status %K WHO %X Not available %B 11th Annual International Conference on Health Economics, Management and Policy %8 0/6/2012 %G eng %N Health Economics,Management and Policy %1 35618 %2 Juin 2012 %0 Generic %D 2012 %T Social challenges facing the cystic fibrosis patients in Belgium on their path to adulthood. %A Simeon Wanyama %K Belgium %K challenge %K conference %K Cystic Fibrosis %K European %K Fibrosis %K IS %K ON %K Patient %K patients %K REGISTRIES %K Registry %K SOCIAL %K WHO %X

Not available

%B 35th European Cystic Fibrosis Conference %I NA %C NA %8 0/6/2012 %G eng %N European Cystic Fibrosis %1 1324 %2 Juin 2012 %0 Report %D 2011 %T The Belgian Cystic Fibrosis Registry Summary Report 2008 %A Muriel Thomas %A Simeon Wanyama %A Herwig Jansens %A François Vermeulen %P 44 %8 2011 %G eng %M D/2011/2505/28 %0 Journal Article %J Eur Respir J %D 2011 %T Inhaled corticosteroids and lower lung function decline in young children with cystic fibrosis. %A K De Boeck %A F Vermeulen %A Simeon Wanyama %A Muriel Thomas %K Administration, Inhalation %K ADOLESCENT %K Adrenal Cortex Hormones %K Belgium %K Child %K Cystic Fibrosis %K Female %K Humans %K Lung %K Male %K Respiratory Function Tests %K Treatment Outcome %K Young adult %X

A recent American registry analysis in cystic fibrosis (CF) children showed less lung function decline after starting inhaled corticosteroid (ICS) use. We therefore examined the influence of ICS treatment on lung function in Belgian CF patients. Data from patients ≥ 6 yrs of age were eligible, provided entries on lung function, height and ICS use were available in two consecutive years. Data after oral steroid use or transplant were excluded. 852 subjects contributed data with 2,976 data pairs analysed, 44.9% concerning years of ICS use. Yearly % predicted decline in forced expiratory volume in 1 s (FEV₁) was 1.07% lower during ICS use (p = 0.001). Subgroup analysis for age revealed that the lower FEV₁ decline rate during ICS use was only statistically significant in children 6-12 yrs of age (2.56%; p = 0.0003). Baseline FEV(1) was lower by 5.89% (p < 0.0001) in ICS users for all age groups combined, but there was no difference in baseline lung function in the children 6-12 yrs of age. In 6-12-yr-old children with CF, baseline lung function was similar in ICS users and nonusers, but annualised FEV₁ decline was 2.56% pred lower in ICS users. Our data therefore support recent American findings.

%B Eur Respir J %V 37 %8 2011 May %G eng %N 5 %R 10.1183/09031936.00077210 %0 Journal Article %J Archives of Public Health %D 2010 %T Clinical influenza surveillance of Influenza A(H1N1) 2009 pandemic through the network of Sentinel General Practitioners36890 %A Viviane Van Casteren %A Karl Mertens %A Jérôme Antoine %A Simeon Wanyama %A Isabelle Thomas %A Nathalie Bossuyt %K 2009 %K Clinical %K general %K GENERAL PRACTITIONER %K general practitioners %K INFLUENZA %K Network %K pandemic %K practitioner %K Practitioners %K sentinel %K Surveillance %X Not available %B Archives of Public Health %V 68 %P 62 - 67 %8 0/0/2010 %G eng %1 36890 %& 62 %R http://dx.doi.org/10.1186%2F0778-7367-68-2-62