Factors that predict short-term intensive care unit mortality in patients with cirrhosis.

BACKGROUND & AIMS: Despite advances in critical care medicine, the mortality rate is high among critically ill patients with cirrhosis. We aimed to identify factors that predict early (7 d) mortality among patients with cirrhosis admitted to the intensive care unit (ICU) and to develop a risk-stratification model. METHODS: We collected data from patients with cirrhosis admitted to the ICU at Indiana University (IU-ICU) from December 1, 2006, through December 31, 2009 (n = 185), or at the University of Pennsylvania (Penn-ICU) from May 1, 2005, through December 31, 2010 (n = 206). Factors associated with mortality within 7 days of admission (7-d mortality) were determined by logistic regression analyses. A model was constructed based on the predictive parameters available on the first day of ICU admission in the IU-ICU cohort and then validated in the Penn-ICU cohort. RESULTS: Median Model for End-stage Liver Disease (MELD) scores at ICU admission were 25 in the IU-ICU cohort (interquartile range, 23-34) and 32 in the Penn-ICU cohort (interquartile range, 26-41); corresponding 7-day mortalities were 28.3% and 53.6%, respectively. MELD score (odds ratio, 1.13; 95% confidence interval [CI], 1.07-1.2) and mechanical ventilation (odds ratio, 5.7; 95% CI, 2.3-14.1) were associated independently with 7-day mortality in the IU-ICU. A model based on these 2 variables separated IU-ICU patients into low-, medium-, and high-risk groups; these groups had 7-day mortalities of 9%, 27%, and 74%, respectively (concordance index, 0.80; 95% CI, 0.72-0.87; P < 10(-8)). The model was applied to the Penn-ICU cohort; the low-, medium-, and high-risk groups had 7-day mortalities of 33%, 56%, and 71%, respectively (concordance index, 0.67; 95% CI, 0.59-0.74; P < 10(-4)). CONCLUSIONS: A model based on MELD score and mechanical ventilation on day 1 can stratify risk of early mortality in patients with cirrhosis admitted to the ICU. More studies are needed to validate this model and to enhance its clinical utility.

A unique posterior segment manifestation of whipple disease.

PURPOSE: To report a novel posterior segment manifestation of Whipple disease. METHOD: Single observational case study. RESULTS: The white crystalline deposit in the retina, choroiditis, and vitritis resolved with treatment of Whipple disease. CONCLUSION: Whipple disease can be associated with panuveitis, multifocal choroiditis, and white crystalline deposits in the anterior segments and retina, a unique clinical finding.

Whipple's disease-associated pulmonary hypertension with positive vasodilator response despite severe hemodynamic derangements.

Pulmonary hypertension (PH) associated with Whipple's disease (WD-PH) is extremely rare, and the underlying pathophysiological processes are incompletely understood. Alterations in hemodynamics can be severe, with right ventricular (RV) dysfunction being common. A case involving a 23-year-old man with WD-PH who exhibited a dramatic vasodilator response during right heart catheterization despite severely altered pulmonary hemodynamics and concomitant RV dysfunction is reported. While the patient's symptoms responded poorly to treatment with nifedipine and sildenafil, significant improvement in dyspnea, RV dysfunction and pulmonary pressures were noted following antibiotic therapy. The present report highlights that despite severely elevated pulmonary artery pressures and RV dysfunction in WD-PH patients, a highly significant vasodilator response and dramatic improvement with antibiotic therapy may be observed. Furthermore, the case highlights the phenomenon of PH in the setting of inflammation, suggesting that adequate control of the inflammatory response can be accompanied by a marked improvement in hemodynamics in certain types of PH.

Acquired weakness, handgrip strength, and mortality in critically ill patients.

RATIONALE: ICU-acquired paresis (ICUAP) is common in survivors of critical illness. There is significant associated morbidity, including prolonged time on the ventilator and longer hospital stay. However, it is unclear whether ICUAP is independently associated with mortality, as sicker patients are more prone and existing studies have not adjusted for this. OBJECTIVES: To test the hypothesis that ICUAP is independently associated with increased mortality. Secondarily, to determine if handgrip dynamometry is a concise measure of global strength and is independently associated with mortality. METHODS: A prospective multicenter cohort study was conducted in intensive care units (ICU) of five academic medical centers. Adults requiring at least 5 days of mechanical ventilation without evidence of preexisting neuromuscular disease were followed until awakening and were then examined for strength. MEASUREMENTS AND MAIN RESULTS: We measured global strength and handgrip dynamometry. The primary outcome was in-hospital mortality and secondary outcomes were hospital and ICU-free days, ICU readmission, and recurrent respiratory failure. Subjects with ICUAP (average MRC score of < 4) had longer hospital stays and required mechanical ventilation longer. Handgrip strength was lower in subjects with ICUAP and had good test performance for diagnosing ICUAP. After adjustment for severity of illness, ICUAP was independently associated with hospital mortality (odds ratio [OR], 7.8; 95% confidence interval [CI], 2.4-25.3; P = 0.001). Separately, handgrip strength was independently associated with hospital mortality (OR, 4.5; 95% CI, 1.5-13.6; P = 0.007). CONCLUSIONS: ICUAP is independently associated with increased hospital mortality. Handgrip strength is also independently associated with poor hospital outcome and may serve as a simple test to identify ICUAP. Clinical trial registered with www.clinicaltrials.gov (NCT00106665).

Newly diagnosed chronic granulomatous disease in a 53-year-old woman with Crohn disease.

BACKGROUND: Chronic granulomatous disease (CGD) is characterized by defective bactericidal activity of white blood cells, specifically, a defect in superoxide production. Patients experience infections, predominantly caused by catalase-positive bacteria and fungal organisms, that may be severe and life-threatening. Most cases of CGD are diagnosed in children; however, it may rarely go undiagnosed until adulthood in individuals with unexplained infections and granulomatous inflammation. OBJECTIVE: To describe an adult with Crohn disease and recurrent infections who was newly diagnosed as having CGD. METHODS: A 53-year-old woman with a history of liver abscesses and Crohn disease presented with Burkholderia cepacia pneumonia and required a right middle lobe resection. Nitroblue tetrazolium test results confirmed the diagnosis of CGD, and Western blot analysis revealed the absence of the 47-phagocyte oxidase protein. Levels of Crohn-associated specific antibodies to Saccharomyces cerevisiae and Escherichia coli outer membrane porin C were elevated. RESULTS: The patient, newly diagnosed as having CGD, was given intravenous trimethoprim-sulfamethoxazole, after which she improved clinically and was discharged from the hospital in stable condition to receive daily oral trimethoprim-sulfamethoxazole treatment. CONCLUSIONS: The concomitant occurrence of Crohn disease and CGD, both characterized by granulomatous inflammation, is noteworthy. This case study demonstrates that CGD should be considered in adults with recurrent infections, especially those caused by catalase-positive organisms, such as B cepacia.