Treatment-Responsive Granulomatous-Lymphocytic Interstitial Lung Disease in a Pediatric Case of Common Variable Immunodeficiency
Granulomatous-Lymphocytic Interstitial Lung disease (GLILD) is a granulomatous and lymphoproliferative condition occurring in ~25% of Common Variable Immunodeficiency (CVID) patients with the highest prevalence in the late teen to young adult years. GLILD was first described in adults and carries a...
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Формат: | Книга |
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Frontiers Media S.A.,
2019-03-01T00:00:00Z.
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LEADER | 00000 am a22000003u 4500 | ||
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001 | doaj_3fdbcedf32b44775bfa3224f4ea509f7 | ||
042 | |a dc | ||
100 | 1 | 0 | |a Robert Tillman |e author |
700 | 1 | 0 | |a R. Paul Guillerman |e author |
700 | 1 | 0 | |a Timothy Trojan |e author |
700 | 1 | 0 | |a Manuel Silva-Carmona |e author |
700 | 1 | 0 | |a Manuel Silva-Carmona |e author |
700 | 1 | 0 | |a Ivan K. Chinn |e author |
245 | 0 | 0 | |a Treatment-Responsive Granulomatous-Lymphocytic Interstitial Lung Disease in a Pediatric Case of Common Variable Immunodeficiency |
260 | |b Frontiers Media S.A., |c 2019-03-01T00:00:00Z. | ||
500 | |a 2296-2360 | ||
500 | |a 10.3389/fped.2019.00105 | ||
520 | |a Granulomatous-Lymphocytic Interstitial Lung disease (GLILD) is a granulomatous and lymphoproliferative condition occurring in ~25% of Common Variable Immunodeficiency (CVID) patients with the highest prevalence in the late teen to young adult years. GLILD was first described in adults and carries a poor prognosis with survival estimated to be reduced by half. Here we report a pediatric case of CVID-associated GLILD that presented with rapid deterioration over 3 months and responded to adult-based treatment with dual chemotherapeutic agents (rituximab and azathioprine), resulting in complete resolution of clinical findings and near complete resolution of radiologic findings. This case highlights the opportunity to achieve a favorable outcome in GLILD following appropriate diagnosis and therapy. | ||
546 | |a EN | ||
690 | |a Granulomatous-Lymphocytic Interstitial Lung disease | ||
690 | |a Common Variable Immunodeficiency | ||
690 | |a chemotherapeutic agents | ||
690 | |a pediatrics | ||
690 | |a treatment | ||
690 | |a Pediatrics | ||
690 | |a RJ1-570 | ||
655 | 7 | |a article |2 local | |
786 | 0 | |n Frontiers in Pediatrics, Vol 7 (2019) | |
787 | 0 | |n https://www.frontiersin.org/article/10.3389/fped.2019.00105/full | |
787 | 0 | |n https://doaj.org/toc/2296-2360 | |
856 | 4 | 1 | |u https://doaj.org/article/3fdbcedf32b44775bfa3224f4ea509f7 |z Connect to this object online. |