Prospective study of quantitative liver MRI in cystic fibrosis: feasibility and comparison to PUSH cohort ultrasound.

BACKGROUND: Pediatric radiologists can identify a liver ultrasound (US) pattern predictive of progression to advanced liver disease. However, reliably discriminating these US patterns remains difficult. Quantitative magnetic resonance imaging (MRI) may provide an objective measure of liver disease in cystic fibrosis (CF). OBJECTIVE: The purpose of this study was to determine if quantitative MRI, including MR elastography, is feasible in children with CF and to determine how quantitative MRI-derived metrics compared to a research US. MATERIALS AND METHODS: A prospective, multi-institutional trial was performed evaluating CF participants who underwent a standardized MRI. At central review, liver stiffness, fat fraction, liver volume, and spleen volume were obtained. Participants whose MRI was performed within 1 year of US were classified by US pattern as normal, homogeneous hyperechoic, heterogeneous, or nodular. Each MRI measure was compared among US grade groups using the Kruskal-Wallis test. RESULTS: Ninety-three participants (51 females [54.8%]; mean 15.6 years [range 8.1-21.7 years]) underwent MRI. MR elastography was feasible in 87 participants (93.5%). Fifty-eight participants had an US within 1 year of MRI. In these participants, a nodular liver had significantly higher stiffness (P<0.01) than normal or homogeneous hyperechoic livers. Participants with a homogeneous hyperechoic liver had a higher fat fraction (P<0.005) than others. CONCLUSION: MR elastography is feasible in children with CF. Participants with a nodular pattern had higher liver stiffness supporting the US determination of advanced liver disease. Participants with a homogeneous hyperechoic pattern had higher fat fractions supporting the diagnosis of steatosis.

Health-related Quality of Life in a Prospective Study of Ultrasound to Detect Cystic Fibrosis-related Liver Disease in Children.

OBJECTIVES: Cystic fibrosis liver disease (CFLD) begins early in life. Symptoms may be vague, mild, or nonexistent. Progressive liver injury may be associated with decrements in patient health before liver disease is clinically apparent. We examined Health-Related Quality of Life (HRQOL) in children enrolled in a multi-center study of CFLD to determine the impact of early CFLD on general and disease-specific QOL. METHODS: Ultrasound (US) patterns of normal (NL), heterogeneous (HTG), homogeneous (HMG), or nodular (NOD) were assigned in a prospective manner to predict those at risk for advanced CFLD. Parents were informed of results. We assessed parent/child-reported (age ≥5 years) HRQOL by PedsQL 4.0 Generic Core and CF Questionnaire-revised (CFQ-R) prior to US and annually. HRQOL scores were compared by US pattern at baseline (prior to US), between baseline and 1 year and at 5 years. Multivariate analysis of variance (MANOVA) with Hotelling-Lawley trace tested for differences among US groups. RESULTS: Prior to US, among 515 participants and their parents there was no evidence that HTG or NOD US was associated with reduced PedsQL/CFQ-R at baseline. Parents of NOD reported no change in PedsQL/CFQ-R over the next year. Child-report PedsQL/CFQ-R (95 NL, 20 NOD) showed improvement between baseline and year 5 for many scales, including Physical Function. Parents of HMG children reported improved CFQ-R scores related to weight. CONCLUSIONS: Early undiagnosed or pre-symptomatic liver disease had no impact on generic or disease-specific HRQoL, and HRQoL was remarkably stable in children with CF regardless of liver involvement.

Computed tomography of pulmonary veins: review of congenital and acquired pathologies.

Newer-generation CT scanners with ultrawide detectors or dual sources offer millisecond image acquisition times and significantly decreased radiation doses compared to historical cardiac CT and CT angiography. This technology is capable of nearly freezing cardiac and respiratory motion. As a result, CT is increasingly used for diagnosing and monitoring cardiac and vascular abnormalities in the pediatric population. CT is particularly useful in the setting of pulmonary vein evaluation because it offers evaluation of the entire pulmonary venous system and lung parenchyma. In this article we review a spectrum of congenital and acquired pulmonary venous abnormalities, including potential etiologies, CT imaging findings and important factors of preoperative planning. In addition, we discuss optimization of CT techniques for evaluating the pulmonary veins.

Non-inferiority of a non-gadolinium-enhanced magnetic resonance imaging follow-up protocol for isolated optic pathway gliomas.

BACKGROUND: Pediatric patients with optic pathway gliomas (OPGs) typically undergo a large number of follow-up MRI brain exams with gadolinium-based contrast media (GBCM), which have been associated with gadolinium tissue retention. Therefore, careful consideration of GBCM use in these children is warranted. OBJECTIVE: To investigate whether GBCM is necessary for OPG MR imaging response assessment using a blinded, non-inferiority, multi-reader study. MATERIALS AND METHODS: We identified children with OPG and either stable disease or change in tumor size on MRI using a regional cancer registry serving the U.S. Pacific Northwest. For each child, the two relevant, consecutive MRI studies were anonymized and standardized into two imaging sets excluding or including GBCM-enhanced images. Exam pairs were compiled from 42 children with isolated OPG (19 with neurofibromatosis type 1), from a population of 106 children with OPG. We included 28 exam pairs in which there was a change in size between exams. Seven pediatric radiologists measured tumor sizes during three blinded sessions, spaced by at least 1 week. The first measuring session excluded GBCM-enhanced sequences; the others did not. The primary endpoint was intra-reader agreement for ≥ 25% change in axial cross-product measurement, using a 12% non-inferiority threshold. RESULTS: Analysis demonstrated an overall 1.2% difference (95% confidence interval, -3.2% to 5.5%) for intra-reader agreement using a non-GBCM-enhanced protocol and background variability. CONCLUSION: A non-GBCM-enhanced protocol was non-inferior to a GBCM-enhanced protocol for assessing change in size of isolated OPGs on follow-up MRI exams.

Heterogeneous Liver on Research Ultrasound Identifies Children with Cystic Fibrosis at High Risk of Advanced Liver Disease: Interim Results of a Prospective Observational Case-Controlled Study.

OBJECTIVE: To assess if a heterogeneous pattern on research liver ultrasound examination can identify children at risk for advanced cystic fibrosis (CF) liver disease. STUDY DESIGN: Planned 4-year interim analysis of a 9-year multicenter, case-controlled cohort study (Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF). Children with pancreatic insufficient CF aged 3-12 years without known cirrhosis, Burkholderia species infection, or short bowel syndrome underwent a screening research ultrasound examination. Participants with a heterogeneous liver ultrasound pattern were matched (by age, Pseudomonas infection status, and center) 1:2 with participants with a normal pattern. Clinical status and laboratory data were obtained annually and research ultrasound examinations biannually. The primary end point was the development of a nodular research ultrasound pattern, a surrogate for advanced CF liver disease. RESULTS: There were 722 participants who underwent screening research ultrasound examination, of which 65 were heterogeneous liver ultrasound pattern and 592 normal liver ultrasound pattern. The final cohort included 55 participants with a heterogeneous liver ultrasound pattern and 116 participants with a normal liver ultrasound pattern. All participants with at least 1 follow-up research ultrasound were included. There were no differences in age or sex between groups at entry. Alanine aminotransferase (42 ± 22 U/L vs 32 ± 19 U/L; P = .0033), gamma glutamyl transpeptidase (36 ± 34 U/L vs 15 ± 8 U/L; P < .001), and aspartate aminotransferase to platelet ratio index (0.7 ± 0.5 vs 0.4 ± 0.2; P < .0001) were higher in participants with a heterogeneous liver ultrasound pattern compared with participants with a normal liver ultrasound pattern. Participants with a heterogeneous liver ultrasound pattern had a 9.1-fold increased incidence (95% CI, 2.7-30.8; P = .0004) of nodular pattern vs a normal liver ultrasound pattern (23% in heterogeneous liver ultrasound pattern vs 2.6% in normal liver ultrasound pattern). CONCLUSIONS: Research liver ultrasound examinations can identify children with CF at increased risk for developing advanced CF liver disease.

Longer upper airway lengths in Robin Sequence: A case-control study using computed tomography.

BACKGROUND: Direct laryngoscopy and intubation are often difficult in children with Robin Sequence. Previous research characterizing anatomic airway differences has focused on parameters influencing airway patency; there is a paucity of data pertaining to intubation trajectories and depth. Such information could impact airway management approaches and decrease the incidence of endotracheal tube malpositioning. AIM: The study goal was to examine whether longitudinal airway parameters pertaining to intubation are different in children with Robin Sequence compared with age-matched controls. METHOD: This case-control study compared patients with RS <4 years of age who had computed tomography scans of the head and neck to age- and sex-matched controls. Measurements were made of the nasopharynx, oropharynx, hypopharynx, tongue, hyoid, and the front teeth to vocal cord, nares to vocal cord, and nasion-basion distances. Statistical analysis was performed using multiple ANCOVA models with the categorical predictor of Robin Sequence vs control and potential covariates including subject height/length, weight, and age. RESULTS: Thirty-three patients with Robin Sequence and 33 control subjects were included. After controlling for subject height/length, mean front teeth to vocal cord distance was 1.2 cm longer (95% CI: 0.9 to 1.6 cm, P < .001) and mean nares to vocal cord distance was 0.8 cm longer (95% CI: 0.4 to 1.2 cm, P < .001) in patients with Robin Sequence than in controls. The tongue was positioned on average 0.5 cm higher (95% CI: 0.3 to 0.8, P < .001) and 0.9 cm more posterior (95% CI: 0.6 to 1.0 cm, P < .001) in cases than in controls. Moreover, in patients with Robin Sequence, the hyoid was positioned on average 0.5 cm more inferiorly (95% CI: 0.2 to 0.8 cm, P < .001) and 0.2 cm more posteriorly (95% CI: 0.1 to 0.4 cm, P < .01) than controls. CONCLUSION: In patients with Robin Sequence under 4 years of age, the mean front teeth to vocal cord distance was found to be 1.2 cm longer while the mean nares to vocal cord distance was found to be 0.8 cm longer controlling for subject length. Clinicians should account for these differences when selecting and placing endotracheal tubes, particularly those with a preformed bend.

Clarifying radiology's role in safety events: a 5-year retrospective common cause analysis of safety events at a pediatric hospital.

BACKGROUND: Common cause analysis of hospital safety events that involve radiology can identify opportunities to improve quality of care and patient safety. OBJECTIVE: To study the most frequent system failures as well as key activities and processes identified in safety events in an academic children's hospital that underwent root cause analysis and in which radiology was determined to play a contributing role. MATERIALS AND METHODS: All safety events involving diagnostic or interventional radiology from April 2013 to November 2018, for which the hospital patient safety department conducted root cause analysis, were retrospectively analyzed. Pareto charts were constructed to identify the most frequent modalities, system failure modes, key processes and key activities. RESULTS: In 19 safety events, 64 sequential interactions were attributed to the radiology department by the patient safety department. Five of these safety events were secondary to diagnostic errors. Interventional radiology, radiography and diagnostic fluoroscopy accounted for 89.5% of the modalities in these safety events. Culture and process accounted for 55% of the system failure modes. The three most common key processes involved in these sequential interactions were diagnostic (39.1%) and procedural services (25%), followed by coordinating care and services (18.8%). The two most common key activities were interpreting/analyzing (21.9%) and coordinating activities (15.6%). CONCLUSION: Proposing and implementing solutions based on the analysis of a single safety event may not be a robust strategy for process improvement. Common cause analyses of safety events allow for a more robust understanding of system failures and have the potential to generate more specific process improvement strategies to prevent the reoccurrence of similar errors. Our analysis demonstrated that the most common system failure modes in safety events attributed to radiology were culture and process. However, the generalizability of these findings is limited given our small sample size. Aligning with other children's hospitals to use standard safety event terminology and shared databases will likely lead to greater clarity on radiology's direct and indirect contributions to patient harm.

Part-Time Pediatric Radiology: The Realities and Perceptions of Part-Time Employment in the Academic Setting.

OBJECTIVE: The goal of this study is to evaluate the perceptions held by full- and part-time academic pediatric radiologists with regard to the value of part-time radiologists, as well as the value placed on the work of part-time colleagues by their departments and institutions. MATERIALS AND METHODS: Two online surveys were distributed to full- and part-time pediatric radiologists via the Society for Pediatric Radiology e-mail list serve. Survey questions evaluated demographic data of both full- and part-time radiologists, as well as the perceptions each group has of part-time employment. RESULTS: Part-time radiologists reported significantly greater work-life balance than did their full-time counterparts and were less likely to report job dissatisfaction. Full- and part-time faculty have comparable levels of perceived departmental contributions. Part-time faculty were more likely to be younger women, early in their careers, and older men nearing retirement. CONCLUSION: Part-time employment provides perceived benefits of increased work-life balance and job satisfaction and is viewed favorably by both full- and part-time radiologists in academic settings.

Surveillance magnetic resonance imaging for isolated optic pathway gliomas: is gadolinium necessary?

BACKGROUND: Pediatric optic pathway gliomas are typically indolent but have a variable clinical course. Treatment is dictated by symptoms and changes on contrast-enhanced MRI examinations. Gadolinium retention in children has motivated parsimonious use of gadolinium-based contrast agents. OBJECTIVES: To determine surveillance MR factors that motivate changes in tumor-directed therapies and extrapolate cost-efficacy of a non-contrast follow-up protocol. MATERIALS AND METHODS: Using an imaging database search we identified children with isolated optic pathway gliomas and ≥3 follow-up contrast-enhanced MRIs. We reviewed medical records and imaging for: (1) coincident changes on contrast-enhanced MRI and tumor-directed therapy, (2) demographics and duration of follow-up, (3) motivations for intervention, (4) assessment of gadolinium-based contrast agents' utility and (5) health care utilization data. We assessed cost impact in terms of relative value unit (RVU) burden. RESULTS: We included 17 neurofibromatosis type 1 (NF1) and 21 non-NF1 patients who underwent a median 16.9 and 24.3 cumulative contrast-enhanced MR exams over 7.7 years and 8.1 years of follow-up, respectively. Eight children (one with NF1) had intervention based on contrast-enhanced MR findings alone. For these eight, increased tumor size was the only common feature, and it was apparent on non-contrast T2 sequences. For the median patient, a non-contrast follow-up protocol could result in 15.9 (NF1) and 23.3 (non-NF1) fewer gadolinium-based contrast agent administrations, and a 39% lower yearly RVU burden. CONCLUSION: Pediatric patients with isolated optic pathway gliomas undergo a large number of routine contrast-enhanced MR follow-up exams. Gadolinium might not be needed for these exams to inform management decisions. Secondary benefits of a non-contrast follow-up protocol include decreased cost and risk to the patient.

Pediatric Abdominal Radiographs: Common and Less Common Errors.

OBJECTIVE: Interpretation of abdominal radiographs of children benefits from a firm knowledge of the congenital anomalies and pathologies unique to this patient population, leveraged by a systematic approach. Interpretive errors place the patients and their families at risk for a delay in diagnosis, unnecessary additional imaging, a potential increase in the radiation burden, and possible psychologic trauma. CONCLUSION: In this article, we describe the common and uncommon potential pitfalls in pediatric abdominal radiography, using several of our own interpretive errors as a framework while providing teaching points to help avoid these mistakes.

Pediatric Chest Radiographs: Common and Less Common Errors.

OBJECTIVE: Radiographic imaging of the pediatric chest presents several unique challenges and nuances, stemming from congenital variants and pathologic processes specific to this population. Errors in interpretation may lead to inappropriate further imaging, incurring additional radiation exposure and cost, as well as psychologic effects on the patients and their families. CONCLUSION: Here, we aim to highlight some common and less common pitfalls in pediatric chest radiography, as well as some tools for avoiding potential mistakes.

Nares-to-carina distance in children: does a 'modified Morgan formula' give useful guidance during nasal intubation?

BACKGROUND: Knowledge of the normal nares-to-carina (NC) distance might prevent accidental bronchial intubation and be helpful when designing preformed endotracheal tubes (ETT). OBJECTIVE: The aim was to measure NC distance and to examine whether a height/length-based 'modified Morgan formula' would give useful guidance for nasotracheal ETT depth positioning. METHODS: Two groups were studied. A younger group consisted of nasally intubated postoperative patients. In these, NC distance was obtained as the sum of ETT length and the distance from the ETT tip to the carina, as measured from an anteroposterior chest X-ray. An older group consisted of children who had undergone computerized tomography (CT) examination including head, neck, and chest. In these, NC was measured directly from the CT image. The modified Morgan formula was derived from the NC vs height/length relationship. RESULTS: Nares-to-carina distance was best predicted by a linear equation based on patient height. The equation in the younger group (1 day-8 years, n = 57) was: NC (cm) = 0.14 × height + 5.8, R(2) = 0.90, and in the older group (2.1-20 years, n = 45): NC (cm) = 0.15 × height + 3.4, R(2) = 0.93. The equation for the groups combined (n = 102) was: NC (cm) = 0.14 × height + 6.2, R(2) = 0.97. Based on the latter equation, a modified Morgan formula was identified as: ETT position at nares in cm = 0.12 × height + 5. If the ETT had been placed as calculated by this formula, the ETT tip would have been at 85 + 5% (mean ± sd) of NC distance, and the ETT tip-to-carina distance would have been 3.1 ± 1.1 cm (range 0-6.6). Bronchial intubation would not have occurred in any child, but a comparison to tracheal length measurements indicates that ETT tip position could be too proximal in some children. CONCLUSION: The study confirms previous reports: NC distance can be well predicted from height/length. A modified Morgan formula might decrease the risk for accidental endobronchial intubation in infants and children, but ETT position need to be confirmed by auscultation or other verification.

Pediatric ovarian torsion: a pictorial review.

Imaging is crucial in expediting the diagnosis and guiding definitive therapy in children with ovarian torsion. This article reviews the multimodality spectrum of imaging findings in pediatric ovarian torsion, focusing primarily on US appearances. We describe predisposing conditions that can lead to torsion, the pathological basis of the radiologic findings in ovarian torsion, and the common diagnostic pitfalls.

Longitudinal features of STIR bright signal in FSHD.

INTRODUCTION: Magnetic resonance imaging of muscle shows short tau-inversion recovery (STIR) brightness in autosomal dominant facioscapulohumeral muscular dystrophy (FSHD1) suggestive of active inflammation/injury. We measured the longitudinal stability/progression of this potential disease biomarker. METHODS: Nine subjects underwent calf MRI imaging over 2 years. Two radiologists evaluated qualitative muscle changes. RESULTS: In 3/9 subjects, calf muscles demonstrated moderate/severe STIR hyperintensity at Time 1 that had progressed to fatty replacement 2 years later (Time 2). In the remaining subjects, moderate/severe muscle STIR abnormalities, when present, were consistent between exams. Mild STIR+ elevations had roughly similar patterns between exams. CONCLUSIONS: Moderate/severe STIR hyperintensities often foreshadow fatty replacement over a 2-year interval. Whether longer time courses are required to observe muscle degeneration and fatty replacement in some subjects remains to be explored.

Callosal alterations in pyridoxine-dependent epilepsy.

AIM: While there have been isolated reports of callosal morphology differences in pyridoxine-dependent epilepsy (PDE), a rare autosomal disorder caused by ALDH7A1 gene mutations, no study has systematically evaluated callosal features in a large sample of patients. This study sought to overcome this knowledge gap. METHOD: Spanning a wide age range from birth to 48 years, corpus callosum morphology and cross-sectional cerebral area were measured in 30 individuals with PDE (12 males, 18 females, median age 3.92y; 25th centile 0.27, 75th centile 15.25) compared to 30 age-matched comparison individuals (11 males, 19 females, median age 3.85y; 25th centile 0.26, 75th centile 16.00). Individuals with PDE were also divided into age groups to evaluate findings across development. As delay to treatment may modulate clinical severity, groups were stratified by treatment delay (less than or greater than 2wks from birth). RESULTS: Markedly reduced callosal area expressed as a ratio of mid-sagittal cerebral area was observed for the entire group with PDE (p<0.001). Stratifying by age (<1y, 1-10y, >10y) demonstrated posterior abnormalities to be a consistent feature, with anterior regions increasingly involved across the developmental trajectory. Splitting the PDE group by treatment lag did not reveal overall or sub-region callosal differences. INTERPRETATION: Callosal abnormalities are a common feature of PDE not explained by treatment lag. Future work utilizing tract-based approaches to understand inter- and intra-hemispheric connectivity patterns will help in the better understanding the structural aspects of this disease.

Liver and heart MR relaxometry in iron loading: reproducibility of three methods.

PURPOSE: To compare the derived T2* values and reproducibility of three methods used to assess iron-loading in heart and liver. MATERIALS AND METHODS: In 23 pediatric patients, liver and cardiac gradient-echo imaging datasets (within-exam repeated sequence pairs) were evaluated. Data analyses compared derived relaxation values (average of pairs) and coefficient of variation (reproducibility of pairs). RESULTS: T2* values showed differences across methods, with pixel-wise mean > average fit > pixel-wise median. Coefficient of variation was found to be lower (better) with pixel-wise median and average fit methods compared to the pixel-wise mean technique. Maximum coefficient of variation values were lowest for the pixel-wise median approach in both the heart and liver. CONCLUSION: Differences in derived T2* values between methods must be considered when comparing values to established magnetic resonance imaging (MRI)-biopsy formulas. The pixel-wise median and average fit methods demonstrate substantial benefits in reproducibility compared to the pixel-wise mean method. Since minimal variation in measurement is critical for patient care, median processing of relaxometry data may be preferable in both tissue types.

Peer review comments augment diagnostic error characterization and departmental quality assurance: 1-year experience from a children's hospital.

OBJECTIVE: The objective of our study was to categorize radiologist peer review comments and evaluate their functions within the context of a comprehensive quality assurance (QA) program. MATERIALS AND METHODS: All randomly entered radiology peer review comments at our institution were compiled over a 1-year period (January 1, 2011, through December 31, 2011). A Web-based commercially available software package was used to query the comments, which were then exported into a spreadsheet. Each comment was then placed into a single most appropriate category based on consensus decision of two board-certified pediatric radiologists. QA scores associated with each comment were recorded. RESULTS: A total of 427 peer review comments were evaluated. The majority of comments (85.9%) were entered voluntarily with QA scores of 1. A classification system was devised that augments traditional error classification. Seven broad comment categories were identified: errors of observation (25.5%), errors of interpretation (5.6%), inadequate patient data gathering (3.7%), errors of communication (9.6%), interobserver variability (21.3%), informational and educational feedback (23.0%), and complimentary (11.2%). CONCLUSION: Comment-enhanced peer review expands traditional diagnostic error classification, may identify errors that were underscored, provides continuous educational feedback for participants, and promotes a collegial environment.

Pediatric CT: strategies to lower radiation dose.

OBJECTIVE: The introduction of MDCT has increased the utilization of CT in pediatric radiology along with concerns for radiation sequelae. This article reviews general principles of lowering radiation dose, the basic physics that impact radiation dose, and specific CT integrated dose-reduction tools focused on the pediatric population. CONCLUSION: The goal of this article is to provide a comprehensive review of the recent literature regarding CT dose reduction methods, their limitations, and an outlook on future developments with a focus on the pediatric population. The discussion will initially focus on general considerations that lead to radiation dose reduction, followed by specific technical features that influence the radiation dose.

Right ventricular apical sequestration: demonstration of a rare variant of double-chambered right ventricle with MRI.

A 15-year-old boy sustained cardiac arrest and was ultimately found to have imaging characteristics consistent with a right ventricular apical sequestration, a sub-variety of double-chambered right ventricle. This is a unique report demonstrating the MRI findings of this rare variation.

Heterogeneous liver on research ultrasound identifies children with cystic fibrosis at high risk of advanced liver disease.

BACKGROUND: This study examines whether heterogeneous (HTG) pattern on liver ultrasound (US) identifies children at risk for advanced cystic fibrosis liver disease (aCFLD). METHODS: Prospective 6-year multicenter case-controlled cohort study. Children with pancreatic insufficient cystic fibrosis (CF) aged 3-12 years without known cirrhosis underwent screening US. Participants with HTG were matched (by age, Pseudomonas infection status and center) 1:2 with participants with normal (NL) US pattern. Clinical status and laboratory data were obtained annually and US bi-annually for 6 years. Primary endpoint was development of nodular (NOD) US pattern consistent with aCFLD. RESULTS: 722 participants underwent screening US, with 65 HTG and 592 NL. Final cohort included 55 HTG and 116 NL with ≥ 1 follow-up US. ALT, AST, GGTP, FIB-4, GPR and APRI were higher, and platelets were lower in HTG compared to NL. HTG had a 9.5-fold increased incidence (95% confidence interval [CI]:3.4, 26.7, p<0.0001, 32.7% vs 3.4%) of NOD versus NL. HTG had a sensitivity of 82% and specificity of 75% for subsequent NOD. Negative predictive value of a NL US for subsequent NOD was 96%. Multivariate logistic prediction model that included baseline US, age, and log(GPR) improved the C-index to 0.90 compared to only baseline US (C-index 0.78). Based on survival analysis, 50% of HTG develop NOD after 8 years. CONCLUSIONS: Research US finding of HTG identifies children with CF with a 30-50% risk for aCFLD. A score based on US pattern, age and GPR may refine the identification of individuals at high risk for aCFLD. CLINICAL TRIAL REGISTRATION: Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF: NCT 01,144,507 (observational study, no consort checklist).