Publications of Céline KEMPENEERS
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See detailDyskinésie Ciliaire Primitive
KEMPENEERS, Céline ULiege; HOUGRAND, Olivier ULiege; POIRRIER, Anne-Lise ULiege

Scientific conference (2021, September 10)

La dyskinésie ciliaire primitive (DCP) est une pathologie génétique rare, caractérisée par des infections respiratoires et ORL chroniques. La maladie peut se manifester dès les premiers mois de vie, mais ... [more ▼]

La dyskinésie ciliaire primitive (DCP) est une pathologie génétique rare, caractérisée par des infections respiratoires et ORL chroniques. La maladie peut se manifester dès les premiers mois de vie, mais elle mettra malheureusement souvent de longues années avant d’être diagnostiquée, souvent vers 8 ou 9 ans. Il se peut même qu’elle ne le soit jamais, évoluant progressivement vers des bronchectasies. C’est que la DCP reste à ce jour peu connue, et donc rarement soupçonnée. Mais c’est aussi que son diagnostic est très complexe et peu standardisé. [less ▲]

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See detailEvidence for ciliary dyskinesia in cystic fibrosis
Bonhiver, Romane ULiege; Bricmont, Noémie ULiege; Pirotte, Maud et al

Conference (2021, September)

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See detailInfluence of storage temperature for respiratory epithelial samples on ciliary functional analysis
Benchimol, Lionel ULiege; Bricmont, Noémie ULiege; BOBOLI, Hedwige ULiege et al

Poster (2021, March 06)

Introduction and Aim: Primary ciliary dyskinesia is characterized by inherited stationary or dyskinetic respiratory cilia. Digital high speed videomicroscopy is highly sensitive and specific for diagnosis ... [more ▼]

Introduction and Aim: Primary ciliary dyskinesia is characterized by inherited stationary or dyskinetic respiratory cilia. Digital high speed videomicroscopy is highly sensitive and specific for diagnosis but lacks standardization. Particularly, the temperature for samples conservation has never been studied. The study goal was to compare ciliary analysis after samples storage at 4°C or 22°C. Agreement between two independent observers was sought as secondary goal. Material and Methods: Ciliated epithelial samples were obtained by inferior turbinate brushing from 13 healthy subjects, divided equally and conserved at 4°C or at 22°C. Digital high speed videomicroscopy assessed ciliary beat frequency (CBF) and percentage of normal ciliary beat pattern (CBP) nine hours after sampling. Results: There was no significant difference when samples were stored at 4°C or 22°C. Median CBF was 13.37 (12,23 –15,37) Hz and 15.24 (12,89 –16,06) Hz at 4°C and 22°C respectively (p = 0.210). Percentage of normal CBP was 81,80% (63,75 – 90,65) and 80,00% (71,70 – 87,30) at 4°C and 22°C respectively (p = 0.910). Moreover, inter observer analysis showed high agreement rate with 93,4% for the CBF and 90.0% for the CBP. Conclusion: This pilot study suggested with a high inter observer agreement rate that the temperature for respiratory ciliated samples conservation had no effect on ciliary function analysis. Larger studies are needed to confirm these preliminary results, notably in pathological conditions. [less ▲]

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See detailPrimary Ciliary Dyskinesia Center in CHU of Liège
KEMPENEERS, Céline ULiege

Speech/Talk (2020)

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See detailPrimary Ciliary Dyskinesia Center in CHU of Liège
KEMPENEERS, Céline ULiege

Speech/Talk (2020)

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See detailDiffuse alveolar haemorrhage in infants: Report of five cases
Gkogkou, Efthymia ULiege; roux, Isabelle; KEMPENEERS, Céline ULiege et al

in Respiratory Medicine Case Reports (2020)

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See detailAvancées thérapeutiques et évolution vers une médecine personnalisée pour les patients atteints de mucoviscidose
BOBOLI, Hedwige ULiege; Pirson, Jessica; SEGHAYE, Marie-Christine ULiege et al

in Revue Médicale de Liège (2020)

Cystic fibrosis is a genetic disorder responsible for the production of a defective transmembrane protein. In recent years, new protein modulators have been developed. They aim to treat the underlying ... [more ▼]

Cystic fibrosis is a genetic disorder responsible for the production of a defective transmembrane protein. In recent years, new protein modulators have been developed. They aim to treat the underlying cause of the disease. The results on the biomarkers of the function of the CFTR protein and on the clinical outcomes are very encouraging. However, there is an individual heterogeneity in the response to modulators within a same genotype. Furthermore, clinical trials focus on the most common mutations in the CFTR gene, in particular DF508. Intestinal organoids, a new model of ex vivo study, could offer a quick approach to increase access to effective treatment for all patients with cystic fibrosis regardless of their CFTR genotype. Organoids could enable personalized treatment of cystic fibrosis. [less ▲]

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See detailPrimary Ciliary Dyskinesia Center in CHU of Liège
KEMPENEERS, Céline ULiege

Speech/Talk (2020)

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See detailDiffuse alveolar hemorrhage in infants : Report of five cases
Gkogkou, Efthymia ULiege; BROUX, Isabelle ULiege; KEMPENEERS, Céline ULiege et al

in Respiratory Medicine Case Reports (2020), 31

Diffuse alveolar hemorrhage (DAH) is a rare life-threatening condition in children. In this entity, the bleeding originates from the pulmonary microvasculature as a result of microvascular damage leading ... [more ▼]

Diffuse alveolar hemorrhage (DAH) is a rare life-threatening condition in children. In this entity, the bleeding originates from the pulmonary microvasculature as a result of microvascular damage leading to blood leakage into the alveolar spaces. DAH can occur as an isolated medical entity or may be associated with other organ system injury or dysfunction. The classic triad of symptoms includes hemoptysis, anemia and diffuse pulmonary infiltrates. Hemoptysis is the usual presenting symptom but is not constant. A variety of diseases is associated with the development of DAH. Current classification organize the etiologies of diffuse alveolar hemorrhage based on the presence of severe immune disorders (such as systemic vasculitis and collagenosis) or non-immunodeficiency disorders (with an identified cardiac or non-cardiac origin, or idiopathic). The five cases of DAH presented in this study were all diagnosed in full-term infants, four males and one female, with normal neonatal adaptation and without family history of notable diseases. In all cases the diagnosis was made between the age of three and eighteen weeks-old. Moreover, all five patients, at the time of diagnosis, presented with hemoptysis, mild or severe dyspnea, anemia and abnormal chest X-rays. Consequently, the diagnosis of DAH was strongly suspected and, eventually, confirmed by bronchoscopy. Additional laboratory tests, as well as selected serologic and radiographic studies were performed in order to identify a specific etiology. The final diagnoses reflect a variety of causes: infections, idiopathic pulmonary hemosiderosis, accidental suffocation and Heiner syndrome. Treatment included oral corticosteroids except from one patient that received antimicrobial therapy. [less ▲]

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See detailThe influence of the conservation of respiratory epithelial samples on ciliary functional analysis
Bricmont, Noémie ULiege; Benchimol, Lionel ULiege; POIRRIER, Anne-Lise ULiege et al

Conference (2020)

Background: Primary ciliary dyskinesia(PCD) is an inherited otosinopulmonary ciliopathy in which respiratory cilia are stationary, or beat in a dyskinetic manner. Digital high speed videomicroscopy(DHSV ... [more ▼]

Background: Primary ciliary dyskinesia(PCD) is an inherited otosinopulmonary ciliopathy in which respiratory cilia are stationary, or beat in a dyskinetic manner. Digital high speed videomicroscopy(DHSV) allows ciliary functional analysis(CFA), including beat frequency(CBF) and pattern(CBP). DHSV is highly sensitive and specific for PCD diagnosis, but lacks standardization and evidence-based data. The technical process of ciliated epithelial samples before CFA varies among studies. Of importance, delay after sampling and temperature for samples conservation may impact CFA. However, these parameters have never been compared. Aims: To evaluate the stability of ciliary function over time when nasal brushing samples are conserved either in the fridge, or at room temperature. Methods: Ciliated epithelial samples were obtained by brushing the inferior nasal turbinate from 5 non-smoking healthy subjects. The samples were divided equally, and conserved either at 4°C or at 22°C. Beating cilia were recorded using DHSV at 37°C immediately(T0, then 9 hours after sampling(T9). CFA was assessed by CBF and the percentage of normal CBP. Results: There was no significant difference between CFA performed immediately or 9 hours after sampling, regardless of the sample storage temperature. Conclusion: This pilot study suggested that the stability of CFA may be preserved for 9 hours at 22°C and at 4°C. Larger studies are needed to confirm these preliminary results, notably in pathological conditions. [less ▲]

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See detailNasal Brushing Sampling and Processing using Digital High Speed Ciliary Videomicroscopy - Adaptation for the COVID-19 Pandemic.
Bricmont, Noémie ULiege; Benchimol, Lionel ULiege; POIRRIER, Anne-Lise ULiege et al

in Journal of visualized experiments : JoVE (2020), (165),

Primary Ciliary Dyskinesia (PCD) is a genetic motile ciliopathy, leading to significant otosinopulmonary disease. PCD diagnosis is often missed or delayed due to challenges with different diagnostic ... [more ▼]

Primary Ciliary Dyskinesia (PCD) is a genetic motile ciliopathy, leading to significant otosinopulmonary disease. PCD diagnosis is often missed or delayed due to challenges with different diagnostic modalities. Ciliary videomicroscopy, using Digital High-Speed Videomicroscopy (DHSV), one of the diagnostic tools for PCD, is considered the optimal method to perform ciliary functional analysis (CFA), comprising of ciliary beat frequency (CBF) and beat pattern (CBP) analysis. However, DHSV lacks standardized, published operating procedure for processing and analyzing samples. It also uses living respiratory epithelium, a significant infection control issue during the COVID-19 pandemic. To continue providing a diagnostic service during this health crisis, the ciliary videomicroscopy protocol has been adapted to include adequate infection control measures. Here, we describe a revised protocol for sampling and laboratory processing of ciliated respiratory samples, highlighting adaptations made to comply with COVID-19 infection control measures. Representative results of CFA from nasal brushing samples obtained from 16 healthy subjects, processed and analyzed according to this protocol, are described. We also illustrate the importance of obtaining and processing optimal quality epithelial ciliated strips, as samples not meeting quality selection criteria do now allow for CFA, potentially decreasing the diagnostic reliability and the efficiency of this technique. [less ▲]

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See detailProtocole de prise en charge de la bronchiolite
KEMPENEERS, Céline ULiege

Speech/Talk (2019)

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See detailTotal absence of the pericardium associated with hypogammaglobulinemia and bronchiectasis in a girl
KINUANI MULASI, Rachel ULiege; BRUYERE, Pierre-Julien ULiege; Schoysman, Laurent et al

in Paediatric Reports (2019), 11(8250),

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See detailPrimary Ciliary Dyskinesia in CHU of Liège
KEMPENEERS, Céline ULiege

Speech/Talk (2019)

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See detailCiliary Functional Analysis: Beating a path towards standardisation
KEMPENEERS, Céline ULiege; Seaton, Claire; Garcia Espinosa, Bernardo et al

Poster (2019, October 18)

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See detailCiliary functional analysis: Beating a path towards standardization.
KEMPENEERS, Céline ULiege; Seaton, Claire; Garcia Espinosa, Bernardo et al

in Pediatric Pulmonology (2019), 54(10), 1627-1638

Primary ciliary dyskinesia is an inherited disorder in which respiratory cilia are stationary, or beat in a slow or dyskinetic manner, leading to impaired mucociliary clearance and significant ... [more ▼]

Primary ciliary dyskinesia is an inherited disorder in which respiratory cilia are stationary, or beat in a slow or dyskinetic manner, leading to impaired mucociliary clearance and significant sinopulmonary disease. One diagnostic test is ciliary functional analysis using digital high-speed video microscopy (DHSV), which allows real-time analysis of complete ciliary function, comprising ciliary beat frequency (CBF) and ciliary beat pattern (CBP). However, DHSV lacks standardization. In this paper, the current knowledge of DHSV ciliary functional analysis is presented, and recommendations given for a standardized protocol for ciliary sample collection and processing. A proposal is presented for a quantitative and qualitative CBP evaluation system, to be used to develop international consensus agreement, and future DHSV research areas are identified. [less ▲]

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See detailChronic cough in Pediatrics: Algorithms
KEMPENEERS, Céline ULiege

Speech/Talk (2018)

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See detailToux chronique chez l’enfant
KEMPENEERS, Céline ULiege

Speech/Talk (2018)

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See detailTo beat, or not to beat, that is question! The spectrum of ciliopathies.
Kempeneers, Céline ULiege; Chilvers, Mark A.

in Pediatric Pulmonology (2018), 53(8), 1122-1129

Cilia are widely distributed throughout the human body, and have numerous roles in physiology, development, and disease. Ciliary ultrastructure is complex, consisting of nine parallel microtubules ... [more ▼]

Cilia are widely distributed throughout the human body, and have numerous roles in physiology, development, and disease. Ciliary ultrastructure is complex, consisting of nine parallel microtubules doublets, with or without motor dynein arms and a central pair of microtubules. Classification of cilia has evolved over time, and currently, four main classes are described: motile and non-motile cilia with a "9 + 2" structure, and motile and non-motile cilia with a "9 + 0" structure, which depend on the presence or absence of dynein arms and a central pair. Ciliopathies are inherited multisystem disorders of cilia, and may present with a varied spectrum of genotypes and phenotypes. Motor and sensory ciliopathies were historically considered as distinct dysfunctions of motile and non-motile cilia, but recent data indicate that the classical features of motor and sensory cilia may overlap. [less ▲]

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