title: Chitotriosidase enzyme activity: is this a possible chronic inflammation marker in children with common variable immunodeficiency and early atherosclerosis?
abstract: Background Common variable immunodeficiency is a rare clinically symptomatic primary immunodeficiency disorder which manifests a wide variability of symptoms, complications. Atherosclerosis in common variable immunodeficiency patients has not been investigated yet contrary to other severe clinical complications. We aimed to investigate the chitotriosidase enzyme's role as an inflammation and atherosclerosis marker in paediatric common variable immunodeficiency patients. Methods Common variable immunodeficiency patients (n = 24) and healthy controls (n = 23) evaluated for chitotriosidase activity with other inflammation markers (hsCRP, myeloperoxidase, serum amyloid A, ferritin), lipid profile and echocardiographic findings (carotid artery intima media thickness - cIMT, brachial artery flow-mediated vazodilatation - FMD%). Results In patients, the mean chitotriosidase activity (8.98 ± 6.28) was significantly higher than the controls (5.17 ± 3.42) ( P = 0.014). Chitotriosidase showed positive relation with hs-CRP ( P = 0.011) and SAA ( P = 0.011) but had no relation with ferritin ( P = 0.155), HDL ( P = 0.152) or LDL-cholesterol ( P = 0.380). Mean cIMT increased in patients compared with the controls ( P < 0.001) but did not show any relation with chitotriosidase ( P = 0.546). FMD% decreased in patients ( P < 0.001) also showing no relation with chitotriosidase ( P = 0.298). Ventricular myocardial performance indexes had no significant difference, but RVEF% decreased in patients ( P = 0.043). Conclusions High chitotriosidase activity in common variable immunodeficiency patients demonstrated in vivo the presence of activated macrophages indicating ongoing inflammation. Echocardiographic diastolic functional deficiency, increased cIMT and decreased FMD% may be accepted as early atherosclerotic findings, but none of them showed relationship with chitotriosidase activities.
authors.affiliation: 1 Department of Pediatrics, Pediatric Biochemistry, Faculty of Medicine, Ege University, Izmir, Turkey.
authors.forename: Elif
authors.initials: E
authors.lastname: Azarsız
authors.affiliation: 2 Department of Pediatric Immunology, Faculty of Medicine, Ege University, Izmir, Turkey.
authors.forename: Neslihan
authors.initials: N
authors.lastname: Karaca
authors.affiliation: 3 Department of Pediatric Cardiology, Faculty of Medicine, Ege University, Izmir, Turkey.
authors.forename: Erturk
authors.initials: E
authors.lastname: Levent
authors.affiliation: 2 Department of Pediatric Immunology, Faculty of Medicine, Ege University, Izmir, Turkey.
authors.forename: Necil
authors.initials: N
authors.lastname: Kutukculer
authors.affiliation: 4 Department of Biochemistry, Faculty of Medicine, Ege University, Izmir, Turkey.
authors.forename: Eser
authors.initials: E
authors.lastname: Sozmen
mesh_terms: D050197:Atherosclerosis / Q000150:complications*; D015415:Biomarkers / Q000378:metabolism; D016022:Case-Control Studies; D002648:Child; D002908:Chronic Disease; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000201:enzymology*; D005260:Female; D006596:Hexosaminidases / Q000378:metabolism*; D006801:Humans; D007249:Inflammation / Q000201:enzymology; D008297:Male
chemical_list: D015415:Biomarkers; D006596:Hexosaminidases; C086400:chitotriosidase
keywords: Primary immunodeficiency; atherosclerosis; chitotriosidase; inflammation