Pulmonary Follow-Up Imaging in Cartilage-Hair Hypoplasia : a Prospective Cohort Study

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http://hdl.handle.net/10138/337729

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Vakkilainen , S , Klemetti , P , Martelius , T , Seppänen , M J R , Mäkitie , O & Toiviainen-Salo , S 2021 , ' Pulmonary Follow-Up Imaging in Cartilage-Hair Hypoplasia : a Prospective Cohort Study ' , Journal of Clinical Immunology , vol. 41 , no. 5 , pp. 1064-1071 . https://doi.org/10.1007/s10875-021-01007-5

Title: Pulmonary Follow-Up Imaging in Cartilage-Hair Hypoplasia : a Prospective Cohort Study
Author: Vakkilainen, Svetlana; Klemetti, Paula; Martelius, Timi; Seppänen, Mikko J. R.; Mäkitie, Outi; Toiviainen-Salo, Sanna
Contributor organization: Research Programs Unit
HUS Children and Adolescents
Children's Hospital
University of Helsinki
Helsinki University Hospital Area
Clinicum
HUS Inflammation Center
Infektiosairauksien yksikkö
Department of Medicine
Lastentautien yksikkö
CAMM - Research Program for Clinical and Molecular Metabolism
Faculty of Medicine
HUS Medical Imaging Center
Date: 2021-07
Language: eng
Number of pages: 8
Belongs to series: Journal of Clinical Immunology
ISSN: 0271-9142
DOI: https://doi.org/10.1007/s10875-021-01007-5
URI: http://hdl.handle.net/10138/337729
Abstract: Cartilage-hair hypoplasia is a syndromic immunodeficiency with short stature, chondrodysplasia, and variable degree of immune dysfunction. Patients with cartilage-hair hypoplasia are prone to recurrent respiratory tract infections, and the prevalence of bronchiectasis ranges from 29 to 52%. Pulmonary complications contribute significantly to the mortality; therefore, regular lung imaging is essential. However, the optimal schedule for repeated lung imaging remains unestablished. We determined the rate and correlates of progression of structural lung changes in a prospectively followed cohort of 16 patients with cartilage-hair hypoplasia. We analyzed clinical, laboratory, and pulmonary functional testing data and performed lung magnetic resonance imaging at a median interval of 6.8 years since previous imaging. Imaging findings remained identical or improved due to disappearance of inflammatory changes in all evaluated patients. Patients with subtle signs of bronchiectasis on imaging tended to have low immunoglobulin M levels, as well as suffered from pneumonia during the follow-up. In conclusion, our results suggest slow if any development of bronchiectasis in selected subjects with cartilage-hair hypoplasia.
Subject: CHH
combined immunodeficiency
lung imaging
MRI
pulmonary complications
RMRP
3121 General medicine, internal medicine and other clinical medicine
Peer reviewed: Yes
Rights: cc_by
Usage restriction: openAccess
Self-archived version: publishedVersion


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