Figure - PMC

Skip to main content

An official website of the United States government

Here's how you know

Here's how you know

Official websites use .gov
A .gov website belongs to an official government organization in the United States.

Secure .gov websites use HTTPS
A lock ( ) or https:// means you've safely connected to the .gov website. Share sensitive information only on official, secure websites.

View full-text article in PMC

. 2018 Nov 13;8:16719. doi: 10.1038/s41598-018-34953-z

Search in PMC
Search in PubMed
View in NLM Catalog
Add to search

© The Author(s) 2018

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

PMC Copyright notice

Pedigree, clinical and immunologic presentation of the two affected siblings. (a) Pedigree of the investigated consanguineous family of the two affected siblings: II.2: index patient; II.3: second affected child. (b) Chest CT scan of patient II.2 at 3.5 years of age with areas of ground glass opacities, air trapping, multiple irregular nodular opacities (tree-in-bud sign), and bilateral bronchiectasis representing lung parenchyma destruction. (c) Serum immunoglobulin levels, (d) absolute numbers of eosinophil, lymphocyte and lymphocyte subsets of patient II.2 (triangles) and patient II.3 (circles) before (open symbols) and after HSCT (black filled symbols) compared to age-related normal range (gray filled area). (e) Representative flow cytometric plots showing CD4⁺ and CD8⁺ T cell subsets assessed by CCR7 and CD45RA expression in patient II.2 and patient II.3 compared to a healthy control. Percentages of naïve T cells (CCR7⁺CD45RA⁺), central memory T cells (CCR7⁺CD45RA⁻), effector memory T cells (CCR7⁻CD45RA⁻) and T_EMRA cells (CCR7⁻CD45RA⁺) of CD4⁺ or CD8⁺ T cells are indicated by numbers in the respective quadrant.