Differences in the in-Hospital Outcomes of Sickle Cell Crisis Among Patients with and without Liver Cirrhosis: A Nationwide in-Patient Analysis

Differences in the In-Hospital Outcomes of Sickle Cell Crisis among Patients with and without Liver Cirrhosis: A Nationwide In-Patient Analysis

Background: Liver disease has a wide range of presentation in patients with Sickle Cell Disease (PMID: 30947870, PMID: 34662016). These presentations can often present as sickle cell crisis or alongside sickle cell crisis (PMID: 37981193, PMID: 23828202). However, there is limited data on the effect of liver cirrhosis on in-hospital sickle cell crisis outcomes in cirrhotic SCD patients. This study sought to assess this complex relationship while hypothesizing poor in-hospital sickle cell crisis outcomes in this patient population.

Methods: A retrospective cohort study was performed using the National Inpatient Sample database for 2021, extracting diagnoses and procedures using the ICD10-CM codes. Inclusion criteria were Principal Diagnosis of Sickle Cell Crisis and age 18 years or older. We divided our population based on the presence or absence of liver cirrhosis. A comparative analysis of population characteristics and outcomes was done between the treatment groups using the T-test for continuous variables and Pearson’s chi-square test or logistic regression for categorical variables. Finally, we conducted a multivariate logistic regression for the primary outcome, adjusting for age, gender, race, socioeconomic status (ZIPINC_QRTL), and comorbidities- heart failure and asthma. The primary outcome was in-patient mortality, and secondary outcomes were vasopressor requirement and mechanical ventilation. Analyses were performed using STATA Basic Edition Version 18.0. Significance was set at <0.05

Results: 79764 hospitalizations for sickle cell crisis were identified, and 884 had a medical history of Liver Cirrhosis. Among patients with cirrhosis, 51.4% were males and 48.6% were females with mean ages of 41.5 and 29.3 years respectively. The mean Charlson comorbidity index was 2.67 and 0.64 among those with and without liver cirrhosis, respectively (p<0.001).

The in-hospital mortality was over ten times higher among patients with a history of cirrhosis compared to those without cirrhosis (2.86% vs 0.27%; Odds ratio 10.8; p<0.001). Unadjusted regression analysis of secondary outcomes revealed cirrhotic patients had higher odds of intubation and mechanical ventilation (OR=13.14, p<0.001) but no statistically significant difference in requirements for vasopressor use (p=0.088).

In a multivariate logistic regression analysis of primary outcome adjusting for age, gender, race, socioeconomic status, and comorbidities (Heart failure and Asthma), liver cirrhosis remained significantly associated with mortality risk (OR = 3.78, p = 0.005).

Conclusion:

From our study, we identified that liver cirrhosis has a significant impact on morbidity and mortality in cirrhotic sickle cell patients and in hospital sickle cell crisis outcomes. Patients with liver cirrhosis and sickle cell crisis had higher chances of intubation and mechanical ventilation, which placed them at an increased mortality risk. As liver cirrhosis is potentially reversible depending on the cause, aggressive management in patients with sickle cell may positively impact their clinical outcome.