LAUSR.org creates dashboard-style pages of related content for over 1.5 million academic articles. Sign Up to like articles & get recommendations!

576: OUTCOMES ANALYSIS OF CHILDREN DIAGNOSED WITH HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN THE PICU

Photo from wikipedia

Critical Care Medicine • Volume 46 • Number 1 (Supplement) www.ccmjournal.org Learning Objectives: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of uncontrolled immune activation with significant risk for morbidity and… Click to show full abstract

Critical Care Medicine • Volume 46 • Number 1 (Supplement) www.ccmjournal.org Learning Objectives: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of uncontrolled immune activation with significant risk for morbidity and mortality from multisystem organ failure. Our aims were to describe a single center experience of HLH in our pediatric intensive care unit (PICU) over a 10-year period and to identify clinical features that may be associated with worse outcomes. Our outcomes included mortality, hospital and ICU length of stay, and functional and cognitive impairments on discharge. Methods: All patients admitted with HLH to our PICU from 2006–2016 were identified utilizing ICD-9 and ICD-10 codes. Each chart was reviewed for demographic information, HLH diagnostic criteria, laboratory data, Pediatric Risk of Mortality Score (PRISM III), clinical features and events of ICU stay, and PICU and hospital length of stay (LOS). Mortality at 1 year and change in functional status score (FSS) from admission to discharge were recorded. Results: There were 40 admissions with 34 unique patients. Familial HLH was diagnosed in 21% of our patients, and of those with secondary HLH, 34% were related to infection, 30% to an inflammatory process, 13% to malignancy, and 17% were unknown. Median age of our patients was 99 months [IQR 27–187]. Median PRISM score on admission was 9 [IQR 7–16]. Median PICU LOS was 6 days [IQR 2–21] and hospital LOS was 22 days [IQR 12– 37]. During their ICU stay 56% of patients received mechanical ventilation, 43% required vasoactives, 18% required continuous renal replacement therapy, and 5.3% received extracorporeal life support. Clinical factors related to increased PICU LOS included PRISM III score (p = 0.019), maximum LDH (p = 0.017), maximum total bilirubin (p = 0.042), need for mechanical ventilation (p = 0.002), vasoactive use (p = 0.02), and secondary infection (p = 0.007). The most common therapies for HLH included steroids (93%), Etoposide (55%) and Anakinra (48%). Overall 1 year mortality was 45%. Of those patients who survived to discharge, there were no acquired functional and cognitive impairments (median ∆ FSS = 0, IQR 0–1). Conclusions: HLH is a disease with high mortality. Multiple factors associated with the disease may predict longer ICU stay; however, for those patients who are treated and survive to discharge there is relatively little morbidity.

Keywords: medicine; mortality; hemophagocytic lymphohistiocytosis; icu stay; picu

Journal Title: Critical Care Medicine
Year Published: 2018

Link to full text (if available)


Share on Social Media:                               Sign Up to like & get
recommendations!

Related content

More Information              News              Social Media              Video              Recommended



                Click one of the above tabs to view related content.