Determinants of mortality for adults with cystic fibrosis admitted in Intensive Care Unit: a multicenter study

BACKGROUND: Intensive care unit (ICU) admission of adults with cystic fibrosis (CF) is controversial because of poor outcome. This appraisal needs re-evaluation following recent changes in both CF management and ICU daily practice. Objectives were to determine long-term outcome of adults with CF admitted in ICU and to identify prognostic factors. METHODS: Retrospective multicenter study of 60 ICU hospitalizations for 42 adult CF patients admitted between 2000 and 2003. Reason for ICU admission, ventilatory support provided and one-year survival were recorded. Multiple logistic analysis was used to determine predictors of mortality. RESULTS: Prior to ICU admission, all patients (mean age 28.1 ± 8 yr) had a severe lung disease (mean FEV(1 )28 ± 12% predicted; mean PaCO(2 )47 ± 9 mmHg). Main reason for ICU hospitalization was pulmonary infective exacerbation (40/60). At admission, noninvasive ventilation was used in 57% of cases and was successful in 67% of patients. Endotracheal intubation was implemented in 19 episodes. Overall ICU mortality rate was 14%. One year after ICU discharge, 10 of the 28 survivors have been lung transplanted. Among recognized markers of CF disease severity, only the annual FEV(1 )loss was associated with a poor outcome (HR = 1.47 [1.18–1.85], p = 0.001). SAPSII (HR = 1.08 [1.03–1.12], p < 0.001) and endotracheal intubation (HR = 16.60 [4.35–63.34], p < 0.001) were identified as strong independent predictors of mortality. CONCLUSION: Despite advanced lung disease, adult patients with CF admitted in ICU have high survival rate. Endotracheal intubation is associated with a poor prognosis and should be used as the last alternative. Although efforts have to be made in selecting patients with CF likely to benefit from ICU resources, ICU admission of these patients should be considered

Caffeine Clearance and Galactose Elimination Capacity as Prognostic Indicators in Fulminant Hepatic-Failure

Caffeine clearance and galactose elimination capacity were evaluated in parallel on admission and during the clinical course of 16 patients with fulminant hepatic failure. On admission, median caffeine clearance and galactose elimination capacity values were less than 10, and 50% of the values in 12 normal controls (< 0.1 versus 1.0 ml/min per kg, P < 0.01 and 3.8 versus 8.3 mg/kg per min, P < 0.05, respectively). There was no significant difference between values in the seven patients who survived and those who died or received transplants (two), although both patients with galactose elimination capacity values below 2.3 mg/kg per min died. Sequential results remained below the lower limit of the reference range in 27 out of 33 (82%) caffeine clearance studies and in 29 out of 33 cases (88%) of galactose elimination capacity determinations. Although the increase in galactose elimination capacity from admission to the final study day was greater in survivors than in those who died, neither galactose elimination capacity nor caffeine clearance results, alone or in combination, were helpful in predicting outcome from fulminant hepatic failure

Use of quantitative liver function tests - caffeine clearance and galactose elimination capacity - after orthotopic liver transplantation

To establish the potential value of quantitative tests of liver function following orthotopic liver transplantation (OLT), a total of 100 determinations of caffeine clearance (CafCl) and galactose elimination capacity (GEC) were made in ten OLT recipients early in the post-operative course (days 2, 4, 6, 8 and 12) and later when clinically stable (3-12 months). Values were compared with a reference range in six normal volunteers in whom it was shown that the standard doses of caffeine (125 mg) and galactose (0.5 g per kg body weight) could be given together without interference. In orthotopic liver transplantation recipients initial GEC and CafCl measurements showed no correlation with peri-operative blood loss, donor ischaemia time, initial bile flow or survival. Throughout the early post-operative period, there were wide inter- and intraindividual variations in both CafCl (17-fold range from 0.16 to 2.7 ml · min-1 · kg-1) and GEC (2.4-fold range from 5.1 to 12 mg · min-1 · kg-1), but the only correlation of test values with standard liver function test results was between GEC and γ-glutamyltranspeptidase. However, GEC values fell by 19% during periods of acute rejection and there was an inverse correlation of GEC with white cell count probably related to sepsis. There was no consistent profile of changing CafCl or GEC values during the post-operative course with median CafCl values (0.87-1.2 ml · min-1 · kg-1) never significantly different from those in volunteers (1.1 ml · min-1 · kg-1) but median GEC results (ranging from 7.3 mg · min-1 · kg-1 at day 12 to 8.2 mg · min-1 · kg-1 at day 4) significantly lower than in normal volunteers (9.5 mg · min-1 · kg-1) at all times including late follow-up (7.7. mg · min-1 · kg-1) when six patients were clinically well. Mean CafCl increased 46% in four patients who were smoking at follow-up, while the introduction of new drug therapies was associated with decreases up to 59% for nine of eleven drugs, with corresponding changes for GEC never exceeding 11%. CafCl and GEC were of little benefit in differential diagnosis of early post-operative complications in this small heterogeneous group of orthotopic liver transplantation recipients, with interindividual variability and susceptibility to modulation by factors other than liver function (e.g., drug ingestion) severely limiting the value of these procedurally complex tests

Associação entre o estado nutricional e a ingestão dietética em pacientes com fibrose cística Association between nutritional status and dietary intake in patients with cystic fibrosis

OBJETIVO: Determinar a relação entre o estado nutricional e a ingestão dietética de pacientes com fibrose cística. MÉTODOS: Estudo transversal com 85 pacientes com fibrose cística entre 6 e 18 anos de idade. A ingestão dietética foi avaliada pelo registro alimentar de 3 dias com a pesagem dos alimentos consumidos. Os desfechos avaliados foram os seguintes indicadores do estado nutricional: percentual da relação peso/estatura (%P/E), percentil do índice de massa corpórea (IMC), escore Z para estatura/idade (E/I) e peso/idade (P/I), e percentual de ingestão dietética comparada a Recommended Dietary Allowances (RDAs). RESULTADOS: A prevalência de pacientes eutróficos foi de 77,7%, considerando o IMC acima do percentil 25 como ponto de corte, e 83,5% estavam acima de 90% do %P/E. A média de ingestão, avaliada em 82 pacientes, foi de 124,5% da RDA. Nas análises de regressão logística univariada, encontrou-se uma associação significativa entre a variável independente ingestão calórica e o desfecho escore Z E/I. O modelo de análise multivariado, elaborado a partir do desfecho escore Z E/I e ajustado para idade, VEF1, colonização por Staphylococcus aureus resistente à meticilina e número de internações hospitalares, demonstrou que um aumento de 1% da ingestão calórica em relação à RDA diminui em 2% a chance de ter déficit de estatura (OR = 0,98; IC95%: 0,96-1,00). A escolaridade materna demonstrou uma associação limítrofe (p = 0,054). CONCLUSÕES: Houve baixa prevalência de desnutrição nesta amostra. O modelo de estudo demonstrou evidências da associação entre a ingestão dietética e o estado nutricional, sendo esta ingestão um fator preditor de crescimento nesses pacientes.<br>OBJECTIVE: To determine the relationship between nutritional status and dietary intake in patients with cystic fibrosis. METHODS: Cross-sectional study involving 85 cystic fibrosis patients between 6 and 18 years of age. Dietary intake was evaluated by the 3-day diet record (weighing the food consumed). The outcome measures were the following nutritional status indicators: weight/height (W/H%) percentage, body mass index (BMI) percentiles, Z score for weight/age (W/A), Z score for height/age (H/A) and percentage of dietary intake compared with the Recommended Dietary Allowance (RDA). RESULTS: The prevalence of well-nourished patients was 77.7%, using BMI above the 25th percentile as the cut-off value, and the W/H% was above 90% in 83.5%. The mean dietary intake, evaluated in 82 patients, was 124.5% of the RDA. In the univariate logistic regression analyses, we found a significant association between the independent variable calorie intake and the Z score for W/A. The multivariate analysis, based on the Z score for H/A and adjusted for FEV1, methicillin-resistant Staphylococcus aureus colonization and number of hospitalizations, demonstrated that a 1% increase in the calorie intake decreases the chance of having short stature by 2% (OR: 0.98; 95% CI: 0.96-1.00). Maternal level of education showed a borderline association (p = 0.054). CONCLUSIONS: The prevalence of malnutrition was low in this sample of patients. The study model demonstrated an association between dietary intake and nutritional status. Dietary intake was a predictive factor of statural growth in patients with cystic fibrosis