for better awarness of cystic fibrosis related glucose abnormalities 3month Progress Report until December 01 2021 Adrienn Kéri About the lecturer ID: 920220
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Slide1
Translational approach for better awarness of cystic fibrosis related glucose abnormalities
3-month Progress Reportuntil December 01, 2021
Adrienn Kéri
Slide2About the lecturerAdrienn Kéri
PhD StudentDepartment:
Centre
for
Translational
Medicine, Semmelweis UniversityHeim Pál National Pediatric InstituteSupervisor(s):Andrea PárniczkyScientific methodology supervisor:Rita NagyStatistician:Zoltán SiposVision: to improve the life quality and extend the life expectancy of cystic fibrosis patients with better clinical assessmentMission: to improve the clinical assessment of cystic fibrosis related glucose abnormalities by applying up to date scientific results Contact:adrikeri@gmail.com
Slide3Cystic fibrosisThe most common autosomal recessive disease 2
Life-shortening disease, affects multiple organs 2
(1:
Yu
et
al
.; Cystic fibrosis; StatPearls-NCBI Booshelf; 2021)(2:DeBoeck et al.; Cystic fibrosis in the year 2020: A disease with a new face; Acta Pediatrica; 2020)(3:Bismuth et al.; Glucose tolerance and insulin secretion, morbidity, and death in patients
with
cystic fibrosis, Journal of Pediatrics; 2008)(4: Pictures:May et al.; Fibrosis of the pancreas in infants and children: An illustrated review of certain clinical features with special emphasis on the pulmonary and cardiac aspects; Journal of Pediatrics; 1949)
Increased life expectancy thanks to new therapeutic options 3Complications come into light (cystic fibrosis related diabetes (CFRD), osteoporosis) 3
„Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon will die.” 1
4
1949
2021
Slide4SPECIFIC GOALSPROJECT LIST
1. Prevalence of abnormal glucose tolerance in patients with cystic fibrosis: a systematic review and meta-analysis Project started:
September
2021
2.
Prevalence
of abnormal glucose tolerance in children with cystic fibrosis - single center prospective cohort analysis (Cystic Fibrosis Related Pancreatic Disorders Registry (CFRPDR)): a cohort analysis Project started: October 2021
Slide5Prevalence of abnormal glucose tolerance in patients with cystic fibrosisProject started:September 2021Planned
submission date:March 2022S
ystematic
review and meta-analysis
Co-investigator
:
Dorina Bajzát, Félix Juhász
1
Slide6BackgroundBy the age of 40 approximately 50% of the patients have cystic fibrosis related diabetes (CFRD) 1
However:
characteristics and dynamics of the progression in AGT status is still unclear
oral glucose tolerance test (OGTT) is recommended only from the age of ten
2
The spectrum of abnormal glucose tolerance (AGT)
in cystic fibrosis (CF) 1Abnl CGM: abnormal home continuous glucose monitoringINDET: indeterminate glycaemiaIGT: impaired glucose toleranceFH: fasting hyperglycaemiaT1D: type 1 diabetes(1:Moran et al.; Epidemiology, pathophysiology, and prognostic implications of cystic fibrosis-related diabetes: a technical review; Diabetes Care; 2010)(2:Saji et al.; Clinical Guidelines:
Care of Children
with Cystic Fibrosis; Royal Brompton Hospital; 2020)
Slide7QuestionHypothesis: AGT is more prevalent under the age of ten in patients with CF
compared to healthy population.Clinical question: What is the prevalence of AGT in different age groups of CF?
Clinical and/or research i
mplication:
Reconsider current guidelines
Treat AGT as early as possible: the role of diet and potentially oral antidiabetics
P general populationE people with CF, no further restrictionC people without CF, no further restriction O AGT prevalence
Slide8Systematic search
Databases: Medline (5210), Embase (7695), Central
(
643
)
Date
of search: October 17, 2021Search key:("cystic fibrosis" OR mucoviscidosis OR CF) AND ("normal glucose tolerance" OR NGT OR "oral glucose tolerance test" OR OGTT OR diabetes OR "cystic fibrosis related diabetes" OR "cystic fibrosis-related diabetes" OR CFRD OR "abnormal glucose metabolism" OR "abnormal glucose tolerance" OR AGT OR "impaired glucose tolerance" OR IGT OR "impaired fasting glucose" OR IFG OR "indeterminate glycaemia" OR "indeterminate glycemia" OR INDET OR hypoglycaemia OR hypoglycemia)
Slide9Flowchart of selection
Cohen’s Kappa: 0,76
Cohen’s Kappa:
Slide10Prevalence of abnormal glucose tolerance in children with cystic fibrosis - single center prospective cohort analysis (Cystic Fibrosis Related Pancreatic Disorders Registry (CFRPDR))Cohort analysis
of 91 cases
Project started:
October
2021
Planned submission date:
May 20222
Slide11QuestionHypothesis: AGT is more prevalent under the age of
ten in patients with CF compared to healthy population.Clinical question:
What is the prevalence of AGT in children with CF?
P
general population
E
children with CFO AGT prevalenceClinical and/or research implication:Earn knowledge about the dynamics of the progression in glucose tolerance status Treat AGT as early as possiblePrevent all the consequences of untreated diabetes via early identification of AGTAim: To assess the prevalence of AGT in our registry patients especially under the age of tenTo identify biomarkers as early predictor of AGT by examining biobank samples
Slide12Characteristics of study cohort
Eligible for further analysis:Patients with cystic fibrosisChildren (under age of 18 at involvement)
Able to undergo OGTT
Patients with previously diagnosed CFRD
CFRPDR
91
patients82 patientsCFRPDR patientsNo. of patientsYear of examination
Slide13OVERVIEWAIMS
1. Prevalence of abnormal glucose tolerance in patients with cystic fibrosis: a systematic review and meta-analysis
Planned
submission
date: March 20222. Prevalence of abnormal glucose tolerance in children with cystic fibrosis - single center prospective cohort analysis (Cystic Fibrosis Related Pancreatic Disorders Registry (CFRPDR)): a cohort
analysis
Planned submission date: May 2022
Slide14Thank you for your attention!“However beautiful the strategy, you should occasionally look at the results.”
Winston Churchill