Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 19 de 19
Filter
1.
Br J Radiol ; 97(1157): 875-881, 2024 May 07.
Article in English | MEDLINE | ID: mdl-38331407

ABSTRACT

Hypertrophic Cardiomyopathy (HCM) is an inherited myocardial disease characterised by left ventricular hypertrophy, which carries an increased risk of life-threatening arrhythmias and sudden cardiac death. The age of presentation and the underlying aetiology have a significant impact on the prognosis and quality of life of children with HCM, as childhood-onset HCM is associated with high mortality risk and poor long-term outcomes. Accurate cardiac assessment and identification of the HCM phenotype are therefore crucial to determine the diagnosis, prognostic stratification, and follow-up. Cardiac magnetic resonance (CMR) is a comprehensive evaluation tool capable of providing information on cardiac morphology and function, flow, perfusion, and tissue characterisation. CMR allows to detect subtle abnormalities in the myocardial composition and characterise the heterogeneous phenotypic expression of HCM. In particular, the detection of the degree and extent of myocardial fibrosis, using late-gadolinium enhanced sequences or parametric mapping, is unique for CMR and is of additional value in the clinical assessment and prognostic stratification of paediatric HCM patients. Additionally, childhood HCM can be progressive over time. The rate, timing, and degree of disease progression vary from one patient to the other, so close cardiac monitoring and serial follow-up throughout the life of the diagnosed patients is of paramount importance. In this review, an update of the use of CMR in childhood HCM is provided, focussing on its clinical role in diagnosis, prognosis, and serial follow-up.


Subject(s)
Cardiomyopathy, Hypertrophic , Magnetic Resonance Imaging , Humans , Cardiomyopathy, Hypertrophic/diagnostic imaging , Child , Prognosis , Magnetic Resonance Imaging/methods , Follow-Up Studies , Disease Progression
3.
Pediatr Radiol ; 53(4): 788-812, 2023 04.
Article in English | MEDLINE | ID: mdl-36843091

ABSTRACT

Rhabdomyosarcoma, although rare, is the most frequent soft tissue sarcoma in children and adolescents. It can present as a mass at nearly any site in the body, with most common presentations in the head and neck, genitourinary tract and extremities. The optimal diagnostic approach and management of rhabdomyosarcoma require a multidisciplinary team with multimodal treatment, including chemotherapy and local therapy. Survival has improved over the last decades; however, further improvement in management is essential with current 5-year overall survival ranging from 35% to 100%, depending on disease and patient characteristics. In the full patient journey, from diagnosis, staging, management to follow-up after therapy, the paediatric radiologist and nuclear physician are essential members of the multidisciplinary team. Recently, guidelines of the European paediatric Soft tissue sarcoma Study Group, the Cooperative Weichteilsarkom Studiengruppe and the Oncology Task Force of the European Society of Paediatric Radiology (ESPR), in an ongoing collaboration with the International Soft-Tissue Sarcoma Database Consortium, provided guidance for high-quality imaging. In this educational paper, given as a lecture during the 2022 postgraduate ESPR course, the multi-disciplinary team of our national paediatric oncology centre presents the journey of two patients with rhabdomyosarcoma and discusses the impact on and considerations for the clinical (paediatric) radiologist and nuclear physician. The key learning points of the guidelines and their implementation in clinical practice are highlighted and up-to-date insights provided for all aspects from clinical suspicion of rhabdomyosarcoma and its differential diagnosis, to biopsy, staging, risk stratification, treatment response assessment and follow-up.


Subject(s)
Rhabdomyosarcoma , Sarcoma , Soft Tissue Neoplasms , Adolescent , Child , Humans , Rhabdomyosarcoma/diagnostic imaging , Rhabdomyosarcoma/therapy , Sarcoma/diagnostic imaging , Sarcoma/therapy , Diagnostic Imaging , Combined Modality Therapy , Soft Tissue Neoplasms/diagnostic imaging , Soft Tissue Neoplasms/therapy , Soft Tissue Neoplasms/pathology
4.
Pediatr Radiol ; 52(1): 122-133, 2022 Jan.
Article in English | MEDLINE | ID: mdl-34729635

ABSTRACT

Hepatic sinusoidal obstruction syndrome, also known as veno-occlusive disease, can occur as a complication of myeloablative chemotherapy, as a result of low-intensity chemotherapy-related liver toxicity or radiotherapy of the liver. Symptoms of sinusoidal obstruction syndrome can range from asymptomatic to liver dysfunction or severe disease with life-threatening acute multi-organ failure. Imaging features can suggest or support this clinical diagnosis. Familiarity with the imaging spectrum of sinusoidal obstruction syndrome is therefore important for both radiologists and clinical oncologists. Here, multi-modality radiologic appearances of sinusoidal obstruction syndrome in pediatric patients are illustrated, including outcome after follow-up.


Subject(s)
Hepatic Veno-Occlusive Disease , Child , Hepatic Veno-Occlusive Disease/chemically induced , Hepatic Veno-Occlusive Disease/diagnostic imaging , Humans
5.
J Pediatr Gastroenterol Nutr ; 71(4): 433-439, 2020 10.
Article in English | MEDLINE | ID: mdl-32947564

ABSTRACT

OBJECTIVES: Disturbances in lipid metabolism play an important role in the pathogenesis of nonalcoholic fatty liver disease (NAFLD). Using lipidomics, an analytical technique that is used to broadly survey lipid metabolism, we searched for biomarkers in plasma that are correlated with the presence of hepatic steatosis in children with obesity. METHODS: Lipidomics was performed in plasma samples of 21 children with obesity in whom steatosis was detected using proton magnetic resonance spectroscopy (H-MRS) and were compared with the lipidome of 21 samples of nonsteatotic subjects with obesity. RESULTS: Forty-two samples were analyzed (57% boys; median age 15 years). A total of 18 lipid classes constituting 839 different lipid species were identified. A statistically significant increase in alkyldiacylglycerol (TG[O]) and phosphatidylethanolamine (PE) species and a significant decrease in alkyl/alkenyl-phosphatidylethanolamine (PE[O]), alkyl/alkenyl-lysophosphatidylethanolamine (LPE[O]) and alkyl/alkenyl-phosphatidylcholine (PC[O]) was observed in children with hepatic steatosis compared with controls. Twelve individual lipid species of 3 lipid classes were significantly increased in steatotic subjects compared with controls. CONCLUSIONS: In this pilot study, we found statistically significant alterations in 5 major lipid classes and 12 individual lipid species in children with steatosis. These might be potential biomarkers for pediatric NAFLD. Lipidomic studies in larger cohorts of children are needed to determine the diagnostic value of these lipids and determine whether results can be generalized for different age groups and ethnic backgrounds.


Subject(s)
Lipidomics , Non-alcoholic Fatty Liver Disease , Adolescent , Biomarkers/metabolism , Child , Female , Humans , Lipid Metabolism , Liver/metabolism , Male , Non-alcoholic Fatty Liver Disease/diagnosis , Non-alcoholic Fatty Liver Disease/metabolism , Pilot Projects
6.
Eur J Pediatr ; 179(12): 1979, 2020 Dec.
Article in English | MEDLINE | ID: mdl-32965578

ABSTRACT

This article originally published with all author names incorrectly listed. All author names have now been transposed and appear correctly above.

7.
Eur J Pediatr ; 179(12): 1969-1977, 2020 Dec.
Article in English | MEDLINE | ID: mdl-32621136

ABSTRACT

Central nervous system (CNS) infections are potentially life threatening in neonates and can lead to the ill-defined diagnosis of ventriculitis. With this study we aimed to explore and describe ventriculitis regarding clinical, microbiological and ultrasonographic characteristics. We performed a retrospective cohort study including all neonates with a culture-proven CNS infection admitted to our tertiary NICU over a 12-year period (2004-2016). For each case clinical data was gathered, and three timed cranial ultrasounds were anonymized and retrospectively reviewed and assessed for signs of ventriculitis. Forty-five patients were included with 9 (20%) diagnosed with ventriculitis. Mortality in both ventriculitis and non-ventriculitis cases was one-third. Patients with pre-existing conditions as post-haemorrhagic hydrocephalus are at risk of developing ventriculitis. Most common pathogens were gram negative bacteria (68.9%). Ultrasonographic signs of ventriculitis developed over time, and interrater agreement was substantial.Conclusion: Neonatal ventriculitis is a serious entity in the continuum of meningitis. Early and correct diagnoses of ventriculitis are both important because of possible persisting or newly developing hydrocephalus or seizures. Sequential imaging should be performed. What is Known: • CNS infections in neonates lead to high mortality and morbidity. • Ventriculitis is a severe complication of meningitis. What is New: • High morbidity; the majority of ventriculitis patients have pre-existing PHVD and develop seizures and hydrocephalus. • Interrater agreement is good; bedside CUS is a useful tool for reaching a sustainable diagnosis of ventriculitis.


Subject(s)
Central Nervous System Infections , Cerebral Ventriculitis , Encephalitis , Meningitis, Bacterial , Anti-Bacterial Agents/therapeutic use , Cerebral Ventriculitis/diagnostic imaging , Cerebral Ventriculitis/epidemiology , Humans , Infant, Newborn , Male , Meningitis, Bacterial/diagnosis , Meningitis, Bacterial/drug therapy , Retrospective Studies
8.
Eur J Pediatr ; 178(6): 863-870, 2019 Jun.
Article in English | MEDLINE | ID: mdl-30903305

ABSTRACT

Alanine aminotransferase (ALT) and ultrasound (US) are the most commonly used tools for detecting non-alcoholic fatty liver disease (NAFLD). No direct comparison of these two modalities in children exists. We aimed to compare head-to-head the diagnostic accuracy of ALT and US and their combination for detecting NAFLD in children with obesity. Ninety-nine children with severe obesity underwent simultaneous serum-ALT and abdominal ultrasound (US steatosis score 0-3). Proton magnetic resonance spectroscopy was used as reference standard for detecting steatosis/NAFLD. ROC curve analyses were performed to determine diagnostic performance and to determine optimum screening cut-points aiming for a specificity ≥ 80%. The area under the ROC (AUROC) of ALT and US were not significantly different (0.74 and 0.70, respectively). At the optimal ALT threshold (≥40 IU/L), sensitivity was 44% and specificity was 89%. At the optimal US steatosis score (≥ 2), sensitivity was 51% and specificity was 80%. Combining ALT and US did not result in better accuracy than ALT or US alone.Conclusion: ALT and US have comparable and only moderate diagnostic accuracy for detecting hepatic steatosis in children with obesity. A stepwise screening strategy combining both methods does not improve diagnostic accuracy. What is Known: • Alanine aminotransferase (ALT) and ultrasound (US) are the most commonly used tools for detecting non-alcoholic fatty liver disease (NAFLD). • ALT and ultrasound have mediocre accuracy in detecting steatosis in children with obesity. What is New: • In a head-to-head comparison, the difference in diagnostic accuracy of ALT and ultrasound in detecting steatosis is not significant. • A stepwise screening strategy combining both methods does not improve diagnostic accuracy.


Subject(s)
Alanine Transaminase/blood , Non-alcoholic Fatty Liver Disease/diagnosis , Ultrasonography/methods , Adolescent , Child , Cross-Sectional Studies , Female , Humans , Magnetic Resonance Spectroscopy/methods , Male , Pediatric Obesity/complications , Predictive Value of Tests , ROC Curve , Severity of Illness Index
9.
Br J Haematol ; 179(4): 635-647, 2017 11.
Article in English | MEDLINE | ID: mdl-28905365

ABSTRACT

Gaucher disease (GD) is a lysosomal storage disorder characterized by the storage of glycosphingolipids in macrophages. Despite effective therapy, residual disease is present in varying degrees and may be associated with late complications, such as persistent bone or liver disease and increased cancer risk. Gaucher macrophages are capable of storing iron and locations of residual disease may thus be detectable with iron imaging. Forty type 1 GD (GD1) patients and 40 matched healthy controls were examined using a whole-body magnetic resonance imaging protocol consisting of standard sequences, allowing analysis of iron content per organ, expressed as R2* (Hz). Median R2* values were significantly elevated in GD1 patients as compared to healthy controls in liver [41 Hz (range 29-165) vs. 38 Hz (range 28-53), P < 0·01], femoral bone marrow [54 Hz (range 37-129) vs. 49 Hz (range 39-69), P = 0·036] and vertebral bone marrow (118 Hz (range 82-210) vs. 105 Hz (range 76-149), P < 0·01). In the spleen, primarily focal Gaucher lesions known as Gaucheroma were found to have increased R2* values. R2* values of liver, spleen and vertebral bone marrow strongly correlated with serum ferritin levels. GD1 patients with persistent hyperferritinaemia demonstrate increased iron levels in liver and bone marrow, which may carry a risk for liver fibrosis and cancer.


Subject(s)
Gaucher Disease/diagnostic imaging , Iron/metabolism , Adult , Aged , Bone Marrow/chemistry , Bone Marrow/pathology , Case-Control Studies , Cohort Studies , Female , Ferritins/blood , Gaucher Disease/pathology , Humans , Iron/analysis , Liver/chemistry , Liver/pathology , Magnetic Resonance Imaging/methods , Male , Middle Aged , Prospective Studies , Spleen/chemistry , Spleen/pathology , Young Adult
10.
Blood Cells Mol Dis ; 60: 49-57, 2016 09.
Article in English | MEDLINE | ID: mdl-27519945

ABSTRACT

In Gaucher disease (GD) imaging of liver and spleen is part of routine follow-up of GD patients. Focal lesions in both liver and spleen are frequently reported at radiological examinations. These lesions often represent benign accumulations of Gaucher cells, so-called "gaucheroma", but malignancies, especially hepatocellular carcinoma, are more frequently found in GD as well. We report the imaging characteristics of all focal lesions in liver and spleen in the Dutch GD cohort. Of the 95 GD1 patients, 40% had focal splenic and/or hepatic lesions, associated with more severe GD. Lesions identified as gaucheroma have variable imaging characteristics: hyper- to hypointense on MRI, hyper- or hypoechoic on US and hypodense on computed tomography (CT). Hepatic lesions were classified as simple cysts or haemangioma based upon imaging characteristics. Focal nodular hyperplasia (FNH), gaucheroma and hepatocellular carcinoma (HCC) could not be distinguished by conventional US, CT or MRI. Growth of these lesions and/or characteristics of HCC on dynamic CT or MRI and pathology was used to identify or rule out HCC. We propose a decision-making algorithm including the use of growth and dynamic CT- or MRI-scanning to characterize lesions.


Subject(s)
Diagnostic Imaging/methods , Gaucher Disease/diagnostic imaging , Liver/pathology , Spleen/pathology , Algorithms , Carcinoma, Hepatocellular/diagnostic imaging , Decision Making, Computer-Assisted , Focal Nodular Hyperplasia/diagnostic imaging , Gaucher Disease/complications , Gaucher Disease/pathology , Humans , Liver Neoplasms/diagnostic imaging , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Ultrasonography
11.
Obesity (Silver Spring) ; 23(6): 1239-43, 2015 Jun.
Article in English | MEDLINE | ID: mdl-25960049

ABSTRACT

OBJECTIVE: Nonalcoholic fatty liver disease (NAFLD) has been recognized as an independent risk factor for cardiovascular disease in adults. It has not been established whether NAFLD is related to early atherosclerotic changes in children. METHODS: In a cross-sectional study, 78 non diabetic, non smoking children with severe obesity were evaluated for NAFLD. Proton magnetic resonance spectroscopy was used to detect liver steatosis and serum ALT was used as a surrogate marker for steatohepatitis. Carotid intima-media thickness (CIMT) and arterial wall stiffness were measured using ultrasound. RESULTS: Steatosis was present in 41 (53%) of subjects. Of these children, 26 out of 41 (63%) had elevated ALT levels. No differences in CIMT and arterial wall stiffness were observed between those without and with steatosis and those with steatosis plus elevated ALT levels [CIMT = 0.47 (±0.06), 0.48 (±0.06) and 0.48 (±0.07) mm, respectively; stiffness = 2.78 (±0.50), 3.00 (±0.81), and 2.90 (±0.78), respectively]. Steatosis and ALT were not correlated to CIMT (r = -0.02 and -0.14, respectively) or arterial wall stiffness (r = 0.13 and -0.11, respectively). CONCLUSIONS: In this study, no relationship between NAFLD and early atherosclerotic changes in children was observed. An atherogenic effect of steatohepatitis (NASH) on pediatric age and long-term atherogenic consequences of simple steatosis cannot be excluded based on this study.


Subject(s)
Atherosclerosis/etiology , Fatty Liver/etiology , Non-alcoholic Fatty Liver Disease/complications , Obesity/complications , Pediatric Obesity/complications , Adolescent , Adult , Atherosclerosis/pathology , Cardiovascular Diseases/diagnosis , Carotid Intima-Media Thickness , Child , Cross-Sectional Studies , Fatty Liver/pathology , Female , Humans , Male , Non-alcoholic Fatty Liver Disease/pathology , Pediatric Obesity/pathology , Proton Magnetic Resonance Spectroscopy , Risk Factors , Vascular Stiffness
12.
Clin Gastroenterol Hepatol ; 13(3): 440-451.e6, 2015 Mar.
Article in English | MEDLINE | ID: mdl-25305349

ABSTRACT

BACKGROUND & AIMS: Magnetic resonance elastography (MRE) is a noninvasive tool for staging liver fibrosis. We conducted a meta-analysis of individual participant data collected from published studies to assess the diagnostic accuracy of MRE for staging liver fibrosis in patients with chronic liver diseases (CLD). METHODS: Through a systematic literature search of multiple databases (2003-2013), we identified studies on diagnostic performance of MRE for staging liver fibrosis in patients with CLD with native anatomy, using liver biopsy as the standard. We contacted study authors to collect data on each participant's age, sex, body mass index (BMI), liver stiffness (measured by MRE), fibrosis stage, staging system used, degree of inflammation, etiology of CLD, and interval between MRE and biopsy. Through a pooled analysis, we calculated cluster-adjusted area under the receiver-operating curve, sensitivity, and specificity of MRE for any fibrosis (≥stage 1), significant fibrosis (≥stage 2), advanced fibrosis (≥stage 3), and cirrhosis (stage 4). RESULTS: We analyzed data from 12 retrospective studies, comprising 697 patients (mean age, 55 ± 13 y; 59.4% male; mean BMI, 26.9 ± 6.7 kg/m(2); 92.1% with <1 year interval between MRE and biopsy; and 47.1% with hepatitis C). Overall, 19.5%, 19.4%, 15.5%, 15.9%, and 29.7% patients had stage 0, 1, 2, 3, and 4 fibrosis, respectively. The mean area under the receiver-operating curve values (and 95% confidence intervals) for the diagnosis of any (≥stage 1), significant (≥stage 2), advanced fibrosis (≥stage 3), and cirrhosis, were as follows: 0.84 (0.76-0.92), 0.88 (0.84-0.91), 0.93 (0.90-0.95), and 0.92 (0.90-0.94), respectively. A similar diagnostic performance was observed in stratified analysis based on sex, obesity, and etiology of CLD. The overall rate of failure of MRE was 4.3%. CONCLUSIONS: Based on a pooled analysis of data from individual participants, MRE has a high accuracy for the diagnosis of significant or advanced fibrosis and cirrhosis, independent of BMI and etiology of CLD. Prospective studies are warranted to better understand the diagnostic performance of MRE.


Subject(s)
Elasticity Imaging Techniques/methods , Liver Cirrhosis/diagnosis , Liver/diagnostic imaging , Liver/pathology , Adult , Aged , Biopsy , Female , Histocytochemistry , Humans , Male , Middle Aged , ROC Curve , Sensitivity and Specificity
13.
Eur Radiol ; 24(3): 638-48, 2014 Mar.
Article in English | MEDLINE | ID: mdl-24158528

ABSTRACT

OBJECTIVE: To compare the diagnostic accuracy of TE and MRE and establish cutoff levels and diagnostic strategies for both techniques, enabling selection of patients for liver biopsy. METHODS: One hundred three patients with chronic hepatitis B or C and liver biopsy were prospectively included. Areas under curves (AUROC) were compared for TE and MRE for METAVIR fibrosis grade ≥ F2 and ≥F3. We defined cutoff values for selection of patients with F0-F1 (sensitivity >95%) and for significant fibrosis F2-F4 (specificity >95%). RESULTS: Following exclusions, 85 patients were analysed (65 CHB, 19 CHC, 1 co-infected). Fibrosis stages were F0 (n = 3), F1 (n = 53), F2 (n = 15), F3 (n = 8) and F4 (n = 6). TE and MRE accuracy were comparable [AUROCTE ≥ F2: 0.914 (95% CI: 0.857-0.972) vs. AUROCMRE ≥ F2: 0.909 (0.840-0.977), P = 0.89; AUROCTE ≥ F3: 0.895 (0.816-0.974) vs. AUROCMRE ≥ F3: 0.928 (0.874-0.982), P = 0.42]. Cutoff values of <5.2 and ≥8.9 kPa (TE) and <1.66 and ≥2.18 kPa (MRE) diagnosed 64% and 66% of patients correctly as F0-F1 or F2-F4. A conditional strategy in inconclusive test results increased diagnostic yield to 80%. CONCLUSION: TE and MRE have comparable accuracy for detecting significant fibrosis, which was reliably detected or excluded in two-thirds of patients. A conditional strategy further increased diagnostic yield to 80%. KEY POINTS: • Both ultrasound-based transient elastography and magnetic resonance elastography can assess hepatic fibrosis. • Both have comparable accuracy for detecting liver fibrosis in viral hepatitis. • The individual techniques reliably detect or exclude significant liver fibrosis in 66 %. • A conditional strategy for inconclusive findings increases the number of correct diagnoses.


Subject(s)
Hepatitis B, Chronic/diagnostic imaging , Hepatitis B, Chronic/pathology , Hepatitis C, Chronic/diagnostic imaging , Hepatitis C, Chronic/pathology , Liver Cirrhosis/diagnostic imaging , Liver Cirrhosis/pathology , Adult , Area Under Curve , Biopsy , Elasticity Imaging Techniques/methods , Female , Hepatitis B, Chronic/complications , Hepatitis C, Chronic/complications , Humans , Liver Cirrhosis/etiology , Magnetic Resonance Imaging/methods , Male , Middle Aged , Prospective Studies , ROC Curve , Sensitivity and Specificity , Young Adult
14.
Abdom Imaging ; 39(2): 283-90, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24366108

ABSTRACT

PURPOSE: MR elastography (MRE) can serve as an accurate surrogate marker of liver fibrosis. For any diagnostic test that is to replace the current reference standard, interobserver agreement should be at least as good and preferably better. The objective of this study was to perform a head-to-head comparison of the interobserver agreements of MRE and liver fibrosis staging on biopsy in a single cohort of hepatitis patients. METHODS: One hundred and three patients with viral hepatitis B or C who had a liver biopsy underwent MRE. Two readers independently selected a region-of-interest (ROI) in the liver to derive elasticity values. Two pathologists first independently staged fibrosis on biopsies using the METAVIR classification and subsequently held a consensus meeting. Interobserver agreements of elasticity values and fibrosis stages were assessed with intraclass correlation coefficients (ICC). RESULTS: MRE and biopsy data were available for 85/103 patients. ICC of pathologists staging fibrosis was almost perfect at 0.91 (95% CI 0.86-0.94). ICC for MRE readers was significantly (P < 0.0001) higher at 0.99 (95% CI 0.98-1.00). CONCLUSIONS: Interobserver agreement for liver fibrosis staging was almost perfect for both histopathology and MRE, with a significant higher agreement for MRE. Its high interobserver agreement and reliable accuracy support the use of MRE as a non-invasive screening tool for liver fibrosis.


Subject(s)
Elasticity Imaging Techniques/methods , Liver Cirrhosis/pathology , Magnetic Resonance Imaging/methods , Biopsy , Female , Hepatitis B/pathology , Hepatitis C/pathology , Humans , Image Interpretation, Computer-Assisted , Liver Cirrhosis/virology , Male , Observer Variation , Retrospective Studies
15.
Radiology ; 269(3): 768-76, 2013 Dec.
Article in English | MEDLINE | ID: mdl-23824991

ABSTRACT

PURPOSE: To define thresholds for detecting significant change in liver viscoelasticity with magnetic resonance (MR) elastography, both for whole-liver measurements and for voxel-wise measurements in relation to spatial resolution. MATERIALS AND METHODS: This prospective study was approved by the institutional review board, and all participants provided written informed consent. Thirty participants (16 volunteers and 14 patients with hepatitis B or C; 18 men; median age, 30.4 years; age range, 18.9-58.6 years) underwent imaging twice while in the same position (intraimage reproducibility), after repositioning (within-day reproducibility), and 1-4 weeks later (between-weeks reproducibility). MR elastography parameters comprised elasticity, viscosity, attenuation parameter α, and propagation parameter ß. Bland-Altman analysis was used to calculate repeatability indexes for each parameter. Analyses were performed in a region-of-interest and a voxel-by-voxel level. Voxel-wise results were calculated in relation to spatial resolution by applying Gaussian filtering to establish the optimal trade-off point between resolution and reproducibility. RESULTS: For elasticity, α, and ß, within-day and between-weeks results were significantly lower than intraimage results (P ≤ .018 for all). Within-day and between-weeks results did not differ significantly. Over-time changes of more than 22.2% for elasticity, 26.3% for viscosity, 26.8% for α, and 10.1% for ß represented thresholds for significant change. The optimal trade-off between spatial resolution and reproducibility was found at a filter size of 8-mm full width at half maximum (FWHM) for elasticity and propagation parameter ß and at 16-mm FWHM for viscosity and attenuation parameter α. CONCLUSION: Repositioning causes a significant decrease in the reproducibility of MR elastography. The propagation parameter ß is the most reliable parameter, with an over-time threshold for significant change of 10.1% and the ability to reproduce viscoelasticity up to a resolution of 8-mm FWHM. Online supplemental material is available for this article.


Subject(s)
Elasticity Imaging Techniques/methods , Hepatitis B/pathology , Hepatitis C/pathology , Adolescent , Adult , Elasticity , Female , Humans , Male , Middle Aged , Patient Positioning , Prospective Studies , Reproducibility of Results , Time Factors , Viscosity
16.
PLoS One ; 8(3): e57507, 2013.
Article in English | MEDLINE | ID: mdl-23554863

ABSTRACT

Long term liver-related complications of type-1 Gaucher disease (GD), a lysosomal storage disorder, include fibrosis and an increased incidence of hepatocellular carcinoma. Splenectomy has been implicated as a risk factor for the development of liver pathology in GD. High ferritin concentrations are a feature of GD and iron storage in Gaucher cells has been described, but iron storage in the liver in relation to liver fibrosis has not been studied. Alternatively, iron storage in GD may be the result of iron supplementation therapy or regular blood transfusions in patients with severe cytopenia. In this pilot study, comprising 14 type-1 GD patients (7 splenectomized, 7 non-splenectomized) and 7 healthy controls, we demonstrate that liver stiffness values, measured by Transient Elastography and MR-Elastography, are significantly higher in splenectomized GD patients when compared with non-splenectomized GD patients (p = 0.03 and p = 0.01, respectively). Liver iron concentration was elevated (>60±30 µmol/g) in 4 GD patients of whom 3 were splenectomized. No relationship was found between liver stiffness and liver iron concentration. HFE gene mutations were more frequent in splenectomized (6/7) than in non-splenectomized (2/7) participants (p = 0.10). Liver disease appeared more advanced in splenectomized than in non-splenectomized patients. We hypothesize a relationship with excessive hepatic iron accumulation in splenectomized patients. We recommend that all splenectomized patients, especially those with evidence of substantial liver fibrosis undergo regular screening for HCC, according to current guidelines.


Subject(s)
Elasticity Imaging Techniques , Gaucher Disease , Iron/metabolism , Liver Cirrhosis , Magnetic Resonance Imaging , Adult , Aged , Carcinoma, Hepatocellular/metabolism , Carcinoma, Hepatocellular/pathology , Carcinoma, Hepatocellular/surgery , Female , Gaucher Disease/diagnostic imaging , Gaucher Disease/metabolism , Gaucher Disease/surgery , Humans , Incidence , Liver Cirrhosis/diagnostic imaging , Liver Cirrhosis/metabolism , Liver Cirrhosis/surgery , Liver Neoplasms/metabolism , Liver Neoplasms/pathology , Liver Neoplasms/surgery , Male , Middle Aged , Radiography , Splenectomy
17.
Obesity (Silver Spring) ; 21(3): 583-90, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23592667

ABSTRACT

BACKGROUND: Accurate prediction scores for liver steatosis are demanded to enable clinicians to noninvasively screen for nonalcoholic fatty liver disease (NAFLD). Several prediction scores have been developed, however external validation is lacking. OBJECTIVE: The aim was to determine the diagnostic accuracy of four existing prediction scores in severely obese children, to develop a new prediction score using novel biomarkers and to compare these results to the performance of ultrasonography. DESIGN AND RESULTS: Liver steatosis was measured using proton magnetic resonance spectroscopy in 119 severely obese children (mean age 14.3 ± 2.1 years, BMI z-score 3.35 ± 0.35). Prevalence of steatosis was 47%. The four existing predictions scores ("NAFLD liver fat score," "fatty liver index," "hepatic steatosis index," and the pediatric prediction score) had only moderate diagnostic accuracy in this cohort (positive predictive value (PPV): 70, 61, 61, 69% and negative predictive value (NPV) 77, 69, 68, 75%, respectively). A new prediction score was built using anthropometry, routine biochemistry and novel biomarkers (leptin, adiponectin, TNF-alpha, IL-6, CK-18, FGF-21, and adiponutrin polymorphisms). The final model included ALT, HOMA, sex, and leptin. This equation (PPV 79% and NPV 80%) did not perform substantially better than the four other equations and did not outperform ultrasonography for excluding NAFLD (NPV 82%). CONCLUSION: The conclusion is in severely obese children and adolescents existing prediction scores and the tested novel biomarkers have insufficient diagnostic accuracy for diagnosing or excluding NAFLD.


Subject(s)
Biomarkers/blood , Fatty Liver/diagnosis , Fatty Liver/epidemiology , Obesity/physiopathology , Adiponectin/blood , Adolescent , Alanine Transaminase/blood , Anthropometry , Body Mass Index , Child , Cholesterol/blood , Fatty Liver/diagnostic imaging , Female , Fibroblast Growth Factors/blood , Humans , Interleukin-6/blood , Leptin/blood , Liver/pathology , Logistic Models , Magnetic Resonance Spectroscopy , Male , Non-alcoholic Fatty Liver Disease , Predictive Value of Tests , Prevalence , Prospective Studies , Triglycerides/blood , Tumor Necrosis Factor-alpha/blood , Ultrasonography
18.
Radiology ; 262(1): 327-34, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22106358

ABSTRACT

PURPOSE: To evaluate the diagnostic accuracy of ultrasonography (US) for the assessment of hepatic steatosis in severely obese adolescents, with proton magnetic resonance (MR) spectroscopy as the reference standard, and to provide insight on the influence of prevalence on predictive values by calculating positive and negative posttest probabilities. MATERIALS AND METHODS: This prospective study was institutional review board approved. All participants, and/or their legal representatives, gave written informed consent. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated for the overall presence of steatosis and for the presence of substantial (moderate to severe) steatosis. Positive and negative posttest probabilities were calculated and plotted against prevalence. RESULTS: A total of 104 children (47 male, 57 female) were prospectively included. Mean age was 14.5 years (range, 8.3-18.9 years) and mean age-adjusted standard deviation body mass index (BMI) score (BMI z score) was 3.3 (range, 2.6-4.1). The overall prevalence of hepatic steatosis was 46.2% (48 of 104). Sensitivity of US was 85.4% (41 of 48), specificity was 55.4% (31 of 56), PPV was 62.1% (41 of 66), and NPV was 81.6% (31 of 38). The prevalence of substantial steatosis was 15.4% (16 of 104), with US sensitivity of 75.0% (12 of 16) and specificity of 87.5% (77 of 88). PPV was 52.2% (12 of 23) and NPV was 95.1% (77 of 81). Plotting of posttest probabilities against prevalence for both disease degrees demonstrated how disease prevalence influences US accuracy. CONCLUSION: Positive US results in severely obese adolescents cannot be used to accurately predict the presence and severity of hepatic steatosis, and additional imaging is required. Negative US results exclude the presence of substantial steatosis with acceptable accuracy. Steatosis prevalence differs among specific populations, strongly influencing posttest probabilities.


Subject(s)
Fatty Liver/diagnostic imaging , Magnetic Resonance Spectroscopy/methods , Obesity/complications , Adolescent , Body Mass Index , Child , Female , Humans , Male , Predictive Value of Tests , Prospective Studies , Sensitivity and Specificity , Statistics, Nonparametric , Ultrasonography
19.
Eur Radiol ; 21(1): 87-97, 2011 Jan.
Article in English | MEDLINE | ID: mdl-20680289

ABSTRACT

OBJECTIVE: To meta-analyse the diagnostic accuracy of US, CT, MRI and 1H-MRS for the evaluation of hepatic steatosis. METHODS: From a comprehensive literature search in MEDLINE, EMBASE, CINAHL and Cochrane (up to November 2009), articles were selected that investigated the diagnostic performance imaging techniques for evaluating hepatic steatosis with histopathology as the reference standard. Cut-off values for the presence of steatosis on liver biopsy were subdivided into four groups: (1) >0, >2 and >5% steatosis; (2) >10, >15 and >20%; (3) >25, >30 and >33%; (4) >50, >60 and >66%. Per group, summary estimates for sensitivity and specificity were calculated. The natural-logarithm of the diagnostic odds ratio (lnDOR) was used as a single indicator of test performance. RESULTS: 46 articles were included. Mean sensitivity estimates for subgroups were 73.3-90.5% (US), 46.1-72.0% (CT), 82.0-97.4% (MRI) and 72.7-88.5% (1H-MRS). Mean specificity ranges were 69.6-85.2% (US), 88.1-94.6% (CT), 76.1-95.3% (MRI) and 92.0-95.7% (1H-MRS). Overall performance (lnDOR) of MRI and 1H-MRS was better than that for US and CT for all subgroups, with significant differences in groups 1 and 2. CONCLUSION: MRI and 1H-MRS can be considered techniques of choice for accurate evaluation of hepatic steatosis.


Subject(s)
Fatty Liver/diagnosis , Liver Neoplasms/diagnosis , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Ultrasonography , Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Humans , Liver Neoplasms/pathology , Male , Meta-Analysis as Topic , Middle Aged , Sensitivity and Specificity
SELECTION OF CITATIONS
SEARCH DETAIL
...