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Objective: Sickle cell disease is associated with a decreased life expectancy, half of the deaths occurring in the ICU. We aimed to describe the characteristics of sickle cell disease patients admitted to ICU and to identify early predictors of a complicated outcome, defined as the need for vital support or death., Design: Retrospective observational cohort study of sickle cell disease patients over a 6-year period., Setting: ICU of a French teaching hospital and sickle cell disease referral center., Patients: Hundred thirty-eight ICU admissions in 119 sickle cell disease patients., Interventions: None., Measurements and Main Results: ICU admission was mainly indicated for sickle cell disease-related events, especially acute chest syndrome. Mechanical ventilation, vasoactive drugs, and renal replacement therapy were administered to 25 (18%), 10 (7%), and 10 (7%) episodes, respectively. The complicated outcome group (n = 28; 20%) was characterized by a more aggressive acute disease within the 48 hours preceding ICU admission, with a higher respiratory rate, a more frequent acute kidney injury, and a more sustained drop of hemoglobin (all p < 0.01). All nine deaths (7%) were sickle cell disease related. None of the sickle cell disease baseline characteristics predicted accurately a complicated outcome. In multivariate analysis, hemoglobin less than or equal to 7.8 g/dL (odds ratio, 3.6; 95% CI, 1.1-11.9), respiratory rate more than or equal to 32 cycles/min (odds ratio, 5.6; 95% CI, 1.8-17.2), and acute kidney injury on ICU admission (odds ratio, 11.5; 95% CI, 2.5-52.6) were independently associated with a complicated outcome., Conclusions: Sickle cell disease patients are at high risk of complications when admitted to the ICU. A sustained drop of hemoglobin, acute respiratory distress, and kidney injury at admission are strong predictors of a complicated outcome.

Background: Non-typhoidal salmonellosis (NTS) often occurs in children with sickle-cell disease (SCD) and remains a significant cause of mortality in developing countries. However, there is lack of reports on the clinical presentation, outcome and complications of NTS in adults with SCD. Methods: We performed a chart review between 2006 and 2016 of adults SCD diagnosed with NTS in 3 referral centers monitoring approximately 3500 SCD adults. Results: Twenty-three episodes of NTS were diagnosed among 22 SCD adults. Diagnosis was challenging: 65% (n = 15/23) of patients presented with vaso-occlusive crisis (VOC) and 30% had no fever. Isolated serotypes were: ser. Enteritidis (n = 8), ser. Typhimurium (n = 6), others (n = 3). We identified two patterns of infections: (1) bacteremic NTS (n = 15) with (n = 9) or without secondary foci of infections (n = 6); (2) non-bacteremic NTS with extra-intestinal foci of infection (n = 8), including primary bones/joints infections (n = 5). Half of patients with osteo-articular localization (n = 6/13) had a previous history of osteonecrosis (n = 2) or osteomyelitis (n = 4) at the same site. Morbidity was high, 6 patients (26%) were admitted to the intensive care unit, 14 patients (61%) required RBC transfusion for VOC. Half of the episodes (n = 12) required surgery (n = 10) or interventional radiology (n = 2) to control the infection. One patient presented a relapse of NTS bacteraemia one year after the first episode. Conclusions: Besides bloodstream infections, clinical presentation of NTS in adults with SCD is non-specific at admission. A triad including bacteraemia, secondary focis of infection and bone localizations was observed in 30% of cases. [ABSTRACT FROM AUTHOR]

Background: Acute chest syndrome (ACS) is the main cause of morbi-mortality in patients with sickle-cell disease in the intensive care unit (ICU). ACS definition encompasses many types of lung damage, making early detection of the most severe forms challenging. We aimed to describe ACS-related lung ultrasound (LU) patterns and determine LU performance to assess ACS outcome. Results: We performed a prospective cohort study including 56 ICU patients hospitalized for ACS in a tertiary university hospital (Paris, France). LU and bedside spirometry were performed at admission (D0) and after 48 h (D2). Complicated outcome was defined by the need for transfusion of ≥ 3 red blood cell units, mechanical ventilation, ICU length-of-stay > 5 days, or death. A severe loss of lung aeration was observed in all patients, predominantly in inferior lobes, and was associated with decreased vital capacity (22 [15–33]% of predicted). The LU Score was 24 [20–28] on D0 and 20 [15–24] on D2. Twenty-five percent of patients (14/56) had a complicated outcome. Neither oxygen supply, pain score, haemoglobin, LDH and bilirubin values at D0; nor their change at D2, differed regarding patient outcome. Conversely, LU re-aeration score and spirometry change at D2 improved significantly more in patients with a favourable outcome. A negative LU re-aeration score at D2 was an independent marker of severity of ACS in ICU. Conclusions: ACS is associated with severe loss of lung aeration, whose resolution is associated with favourable outcome. Serial bedside LU may accurately and early identify ACS patients at risk of complicated outcome. [ABSTRACT FROM AUTHOR]

Background: Low plasma sodium concentration has been recognized as a prognostic factor in several disorders but never evaluated in sickle cell disease. The present study evaluates its value at admission to predict a complication in adult patients with sickle cell disease hospitalized for an initially uncomplicated acute painful episode.Methods: The primary outcome of this retrospective study, performed between 2010 and 2015 in a French referral center for sickle cell disease, was a composite criterion including acute chest syndrome, intensive care unit transfer, red blood cell transfusion or inpatient death. Analyses were adjusted for age, sex, hemoglobin genotype and concentration, lactate dehydrogenase (LDH) concentration, and white blood cell count.Results: We included 1218 stays (406 patients). No inpatient death occurred during the study period. Hyponatremia (plasma sodium ≤135 mmol/L) at admission in the center was associated with the primary outcome (adjusted odds ratio [OR] 1.95, 95% confidence interval [CI] 1.3-2.91, P = 0.001), with acute chest syndrome (OR 1.95 [95% CI 1.2-3.17, P = 0.008]), and red blood cell transfusion (OR 2.71 [95% CI 1.58-4.65, P <0.001]) but not significantly with intensive care unit transfer (OR 1.83 [95% CI 0.94-3.79, P = 0.074]). Adjusted mean length of stay was longer by 1.1 days (95% CI 0.5-1.6, P <0.001) in patients with hyponatremia at admission.Conclusions: Hyponatremia at admission in the medical department for an acute painful episode is a strong and independent prognostic factor of unfavorable outcome and, notably, acute chest syndrome. It could help targeting patients who may benefit from closer monitoring. [ABSTRACT FROM AUTHOR]