Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study.

ABSTRACT:

Background

Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed.

Methods

Among 1482 children (< 15 years) registered in the French LCH registry (1994-2018), 111 (7.4%) had lung involvement. This retrospective study included data for 17 (1.1%) patients that required one or more intensive care unit (ICU) admissions for respiratory failure.

Results

The median age was 1.3 years at the first ICU hospitalization. Of the 17 patients, 14 presented with lung involvement at the LCH diagnosis, and 7 patients (41%) had concomitant involvement of risk-organ (hematologic, spleen, or liver). Thirty-five ICU hospitalizations were analysed. Among these, 22 (63%) were secondary t

SUBMITTER: Le Louet S 

PROVIDER: S-EPMC7487928 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature