Cystic fibrosis-related liver disease is an independent risk factor for mortality and increased health care resource utilization in hospitalized pediatric patients with cystic fibrosis.

Λεπτομέρειες βιβλιογραφικής εγγραφής
Τίτλος:	Cystic fibrosis-related liver disease is an independent risk factor for mortality and increased health care resource utilization in hospitalized pediatric patients with cystic fibrosis.
Συγγραφείς:	Thavamani A; Division of Pediatric Gastroenterology, Hepatology and Nutrition, UH Rainbow Babies and Children's Hospital/Case Western Reserve University School of Medicine, Cleveland, Ohio, USA., Umapathi KK; Division of Pediatric Cardiology, Rush University Medical Center, Chicago, Illinois, USA., Kutney K; Division of Pediatric Endocrinology, UH Rainbow Babies and Children's Hospital/Case Western Reserve University School of Medicine, Cleveland, Ohio, USA., Sferra TJ; Division of Pediatric Gastroenterology, Hepatology and Nutrition, UH Rainbow Babies and Children's Hospital/Case Western Reserve University School of Medicine, Cleveland, Ohio, USA., Sankararaman S; Division of Pediatric Gastroenterology, Hepatology and Nutrition, UH Rainbow Babies and Children's Hospital/Case Western Reserve University School of Medicine, Cleveland, Ohio, USA.
Πηγή:	Pediatric pulmonology [Pediatr Pulmonol] 2022 Jul; Vol. 57 (7), pp. 1717-1725. Date of Electronic Publication: 2022 May 17.
Τύπος έκδοσης:	Journal Article; Research Support, Non-U.S. Gov't
Γλώσσα:	English
Στοιχεία περιοδικού:	Publisher: Wiley-Liss Country of Publication: United States NLM ID: 8510590 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1099-0496 (Electronic) Linking ISSN: 10990496 NLM ISO Abbreviation: Pediatr Pulmonol Subsets: MEDLINE
Imprint Name(s):	Publication: <2005-> : Hoboken, NJ : Wiley-Liss Original Publication: [Philadelphia, PA] : W.B. Saunders, [c1985-
Ιατρικοί όροι (MeSH):	Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Liver Diseases/epidemiology , Liver Diseases/etiology, Liver Cirrhosis/complications ; Liver Cirrhosis/diagnosis ; Child ; Delivery of Health Care ; Humans ; Risk Factors
Περίληψη:	Background: Cystic fibrosis-related liver disease (CFLD) is more prevalent in recent decades due to the increasing life expectancy of patients with cystic fibrosis (CF). There is paucity of population-level data on the impact of CFLD on hospital outcomes. Methods: We interrogated nonoverlapping years (2003-2016) of the National Inpatient Sample and Kids' Inpatient Database to include all hospitalized patients <21 years of age with a primary diagnosis of CF within the United States. A concomitant diagnosis of cirrhosis, liver fibrosis, chronic liver disease, portal hypertension, hepatomegaly, splenomegaly, hypersplenism, and liver transplant status was considered as surrogates for the diagnosis of CFLD and was compared with CF-related hospitalizations without these diagnoses (controls) for demographics, comorbid conditions, in-hospital mortality, length-of-stay, and hospital charges. Results: We evaluated 94,374 CF-related hospitalizations. The prevalence of CFLD was 5.8%. The prevalence increased from 3.1% (2003) to a peak of 7.3% (2014) with an overall increasing trend, p < 0.001. Hospitalizations with CFLD had an increased prevalence of significant comorbidities: respiratory failure,lung transplant, pulmonary hypertension, diabetes mellitus, malnutrition, Clostridioides difficile infection, cholelithiasis, anemia, and need for parenteral nutrition, p < 0.001. Multivariate regression models showed CFLD as independently associated with 2.1 (95% confidence interval [CI]: 1.5 to 2.8) times increased risk of inpatient mortality, contributed to 1.1 (95% CI: 0.89 to 1.37) additional days of hospitalization, and incurring $14,852 (95% CI: 12,204 to 17,501) excess hospital charges, p < 0.001. Conclusion: CFLD is associated with multiple comorbidities and is independently associated with increased risk of mortality and increased health care resource utilization in pediatric CF-related hospitalizations. (© 2022 Wiley Periodicals LLC.)
References:	Kamal N, Surana P, Koh C. Liver disease in patients with cystic fibrosis. Curr Opin Gastroenterol. 2018;34(3):146-151. Leung DH, Narkewicz MR. Cystic fibrosis-related cirrhosis. J Cyst Fibros. 2017;16:S50-S61. Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology. 2002;36(6):1374-1382. Stonebraker JR, Ooi CY, Pace RG, et al. Features of severe liver disease with portal hypertension in patients with cystic fibrosis. Clin Gastroenterol Hepatol. 2016;14(8):1207-1215.e3. Flass T, Narkewicz MR. Cirrhosis and other liver disease in cystic fibrosis. J Cyst Fibros. 2013;12(2):116-124. Rowland M, Gallagher C, Gallagher CG, et al. Outcome in patients with cystic fibrosis liver disease. J Cyst Fibros. 2015;14(1):120-126. Rowland M, Gallagher CG, Ó'laoide R, et al. Outcome in cystic fibrosis liver disease. Am J Gastroenterol. 2011;106(1):104-109. Thornton C, Somayaji R, Parkins M, et al. Characteristics and outcomes of children with cystic fibrosis hospitalized with cirrhosis in the united states. Am J Gastroenterol. 2021;116(8):1734-1737. Corbett K, Kelleher S, Rowland M, et al. Cystic fibrosis-associated liver disease: a population-based study. J Pediatr. 2004;145(3):327-332. Agency for Healthcare Research and Quality. HCUP databases. Healthcare cost and utilization project (HCUP). 2022. Blower K, Seifi A, Michalek J, Keyt H. Incidence of vte in patients with cystic fibrosis: are they high risk? Chest. 2016;150(4):1135A. Chatterjee K, Goyal A, Shah N, Kakkera K, Jagana R, Anderson P. Contemporary national trends of cystic fibrosis hospitalizations and co-morbidities in the united states. Adv Resp Med. 2016;84(6):316-323. Nguyen N, Crealey G, O'Neill C. The impact of modulator therapy in cystic fibrosis on patterns of hospitalization related to age. Results from an analysis of us inpatient episodes 2006-2016. J Cyst Fibros. 2021;20(6):e114-e117. Bell SC, Mall MA, Gutierrez H, et al. The future of cystic fibrosis care: a global perspective. Lancet Respir Med. 2020;8(1):65-124. Cohen-Cymberknoh M, Shoseyov D, Kerem E. Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am J Respir Crit Care Med. 2011;183(11):1463-1471. Stephenson AL, Tom M, Berthiaume Y, et al. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. Eur Respir J. 2015;45(3):670-679. Arnon R, Annunziato RA, Miloh T, et al. Liver and combined lung and liver transplantation for cystic fibrosis: analysis of the unos database. Pediatr Tranplant. 2011;15(3):254-264. Colombo C, Apostolo MG, Ferrari M, et al. Analysis of risk factors for the development of liver disease associated with cystic fibrosis. J Pediatr. 1994;124(3):393-399. Lamireau T, Monnereau S, Martin S, Marcotte J-E, Winnock M, Alvarez F. Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol. 2004;41(6):920-925. Ling S, Wilkinson J, Hollman A, McColl J, Evans T, Paton J. The evolution of liver disease in cystic fibrosis. Arch Dis Child. 1999;81(2):129-132. Scott-Jupp R, Lama M, Tanner M. Prevalence of liver disease in cystic fibrosis. Arch Dis Child. 1991;66(6):698-701. Toledano M, Mukherjee S, Howell J, et al. The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study. PLoS One. 2019;14(4):e0212779. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Registry 2019 Annual Data Report, 2020:92. Kulich M, Rosenfeld M, Goss CH, Wilmott R. Improved survival among young patients with cystic fibrosis. J Pediatr. 2003;142(6):631-636. Rosenfeld M, Davis R, FitzSimmons S, Pepe M, Ramsey B. Gender gap in cystic fibrosis mortality. Am J Epidemiol. 1997;145(9):794-803. Singh H, Coffey MJ, Ooi CY. Cystic fibrosis-related liver disease is associated with increased disease burden and endocrine comorbidities. J Pediatr Gastroenterol Nutr. 2020;70(6):796-800. Leeuwen L, Magoffin AK, Fitzgerald DA, Cipolli M, Gaskin KJ. Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes. Arch Dis Child. 2014;99(5):443-447. Culhane S, George C, Pearo B, Spoede E. Malnutrition in cystic fibrosis: a review. Nutr Clin Pract. 2013;28(6):676-683. https://www.cff.org/Care/Clinical-Care-Guidelines/Other-CF-Related-Conditions-Clinical-Care-Guidelines/Liver-Disease-Clinical-Care-Guidelines/. Dhochak N, Jat KR, Sankar J, Lodha R, Kabra SK. Predictors of malnutrition in children with cystic fibrosis. Indian Pediatr. 2019;56(10):825-830. Colombo C. Liver disease in cystic fibrosis. Curr Opin Pulm Med. 2007;13(6):529-536. Ouyang L, Grosse SD, Amendah DD, Schechter MS. Healthcare expenditures for privately insured people with cystic fibrosis. Pediatr Pulmonol. 2009;44(10):989-996. Van Gool K, Norman R, Delatycki MB, Hall J, Massie J. Understanding the costs of care for cystic fibrosis: an analysis by age and health state. Value Health. 2013;16(2):345-355.
Contributed Indexing:	Keywords: children; chronic liver disease; cystic fibrosis; health care utilization; mortality; population-based study
Entry Date(s):	Date Created: 20220502 Date Completed: 20220624 Latest Revision: 20220715
Update Code:	20260130
DOI:	10.1002/ppul.25941
PMID:	35499220
Βάση Δεδομένων:	MEDLINE

Περιγραφή
ISSN:	1099-0496
DOI:	10.1002/ppul.25941