The prevalence of obesity in patients with cystic fibrosis (CF) is increasing and around one-third of adults with CF are now overweight or obese. The causes of excess weight gain in CF are likely multifactorial, including: adherence to the high-fat legacy diet, reduced exercise tolerance, therapeutic advances, and general population trends. Increased weight has generally been considered favorable in CF, correlating with improved pulmonary function and survival. While the optimal BMI for overall health in CF is unknown, most studies demonstrate minimal improvement in pulmonary function when BMI exceeds 30 kg/m 2 . Dyslipidemia and cardiovascular disease are important co-morbidities of obesity in the general population, but are uncommon in CF. In people with CF, obesity is associated with hypertension and higher cholesterol levels. With longer life expectancy and rising obesity rates, there may be an increase in cardiovascular disease among people with CF in coming years. Overweight CF patients are more likely to be insulin resistant, taking on features of type 2 diabetes. Treating obesity in people with CF requires carefully weighing the metabolic risks of overnutrition with the impact of low or falling BMI on lung function. This article describes current knowledge on the epidemiology, causes, consequence, and treatment of obesity in people with CF.
M R fingerprinting (MRF) provides rapid and simultaneous quantification of multiple tissue parameters (eg, T1 and T2) in a single scan (1-7). The resultant parametric maps are inherently coregistered, enabling a more comprehensive evaluation of tissue composition and pathophysiologic characteristics (1). As MRF has also shown improved reproducibility over a range of magnetic field strengths and system manufacturers, these multiparametric MRF assessments may provide important outcome measures for clinical trials of therapeutics (1,3,(8)(9)(10). While the majority of prior MRF studies are focused on brain imaging, recent work shows that MRF can also generate quantitative T1 and T2 maps of abdominal organs, including the kidney (4). One important requirement of an abdominal MRF acquisition is a short acquisition time, preferably within a single breath hold, to limit respiratory motion artifacts. An improved MRF acquisition would also limit errors in T1 and T2 from known B 1 inhomogeneities (4,8).In this prospective study, we describe a two-dimensional kidney MRF technique that combines multiple features of prior MRF implementations in the brain and heart for improved T1 and T2 mapping in the human kidney at 3.0 T. These features include multiple magnetization preparation schema to provide MRF images with both T1 and T2 contrast enhancement, low flip angles (range, 5°-12°) to limit the impact of B 1 heterogeneities, and a constant repetition time and echo time to provide accurate, precise, and coregistered T1 and T2 maps in 15 seconds without the need for additional B 1 correction (2,5,6). We evaluated this rapid MRF acquisition both in vitro using standardized phantoms and in vivo in the kidneys of healthy volunteers as well as three pediatric patients with autosomal recessive polycystic kidney disease (ARPKD).Background: MR fingerprinting (MRF) provides rapid and simultaneous quantification of multiple tissue parameters in a single scan.Purpose: To evaluate a rapid kidney MRF technique at 3.0 T in phantoms, healthy volunteers, and patients. Materials and Methods:A 15-second kidney MRF acquisition was designed with 12 acquisition segments, a range of low flip angles (5°-12°), multiple magnetization preparation schema (T1, T2, and fat suppression), and an undersampled spiral trajectory. This technique was first validated in vitro using standardized T1 and T2 phantoms. Kidney T1 and T2 maps were then obtained for 10 healthy adult volunteers (mean age 6 standard deviation, 35 years 6 13; six men) and three pediatric patients with autosomal recessive polycystic kidney disease (ARPKD) (mean age, 10 years 6 3; two boys) between August 2019 and October 2020 to evaluate the method in vivo.Results: Results in nine phantoms showed good agreement with spin-echo-based T1 and T2 values (R 2 . 0.99). In vivo MRF kidney T1 and T2 assessments in healthy adult volunteers (cortex: T1, 1362 msec 6 5; T2, 64 msec 6 5; medulla: T1, 1827 msec 6 94; T2, 69 msec 6 3) were consistent with values in the literature but with improved precision...
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)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(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.
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