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Background Despite a proven role in the characterization of liver lesions, use of the gadolinium-based contrast agent (GBCA) gadoxetate disodium at MRI is limited in children due to a lack of comparative safety data. Purpose To evaluate the safety of the GBCA gadoxetate disodium (a linear ionic hepatobiliary contrast agent [HBA]) in children and adolescents, compared with extracellular contrast agents (ECA). Materials and Methods A retrospective analysis was conducted in children and adolescents aged 18 years or younger who underwent HBA-enhanced MRI at one of three tertiary hospitals from January 2010 to December 2022. The incidence of GBCA-associated acute adverse events was compared between MRI examinations with a HBA and those with ECA. Severity was categorized according to American College of Radiology guidelines (mild, moderate, or severe). (a) Propensity score matching using multivariable logistic regression models and (b) inverse probability of treatment weighting analysis based on nine covariates (age, sex, asthma, allergic rhinitis, chronic urticaria or atopy, food allergy, drug allergy, premedication, and history of GBCA-associated adverse events) were used for confounder adjustment. Results A total of 1629 MRI examinations (ECA, n = 1256; HBA, n = 373) in 1079 patients were included (mean age, 8.6 years ± 6.5; 566 girls). The per-examination incidence of GBCA-associated acute adverse events showed no evidence of a difference, with rates of 0.9% (11 of 1256 examinations) for ECA and 1.3% (five of 373 examinations) for HBA (odds ratio [OR], 1.55 [95% CI: 0.54, 4.46]; P = .42). Acute adverse events were all mild with ECA, whereas with HBA, they were mild for four patients and moderate for one patient. There was no evidence of a difference in the incidence of acute adverse events, even in propensity score matching (OR, 1.33 [95% CI: 0.30, 5.96]; P = .71) and inverse probability of treatment weighting analysis (OR, 0.84 [95% CI: 0.25, 2.86]; P = .78). Conclusion Gadoxetate disodium showed no difference in acute adverse events compared with ECA in children and adolescents, with further large-scale pediatric studies required to confirm its safety. © RSNA, 2024 Supplemental material is available for this article. See also the editorial by Otero in this issue.
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Meios de Contraste , Gadolínio DTPA , Imageamento por Ressonância Magnética , Humanos , Masculino , Feminino , Criança , Adolescente , Estudos Retrospectivos , Imageamento por Ressonância Magnética/métodos , Meios de Contraste/efeitos adversos , Pré-Escolar , Fígado/diagnóstico por imagem , Lactente , Hepatopatias/diagnóstico por imagemRESUMO
OBJECTIVES: To evaluate the diagnostic performance of 2021 K-TIRADS biopsy criteria for detecting malignant thyroid nodules in a pediatric population, making comparisons with 2016 K-TIRADS. METHODS: This retrospective study included pediatric patients with histopathologically confirmed diagnoses. The diagnostic performance of 2021 K-TIRADS was compared with that of 2016 K-TIRADS. Simulation studies were performed by changing biopsy cut-off sizes for K-TIRADS 5 to 1.0 cm (K-TIRADS5-1.0cm) and 0.5 cm (K-TIRADS5-0.5cm), and for K-TIRADS 4 to 1.0 cm (K-TIRADS4-1.0cm) and 1.0-1.5 cm (K-TIRADS4-1.0~1.5cm). Subgroup analysis was performed in small (< 1.5 cm) and large nodules (≥ 1.5 cm). RESULTS: Two hundred seventy-seven thyroid nodules (54.9% malignant) from 221 pediatric patients were analyzed. All simulated 2021 K-TIRADS showed higher accuracy than 2016 K-TIRADS. Compared with 2021 K-TIRADS5-1.0cm, 2021 K-TIRADS5-0.5cm showed lower specificity (51.6% vs. 47.9%; p = 0.004) but higher sensitivity (77.2% vs. 90.3%; p < 0.001) and accuracy (62.7% vs. 68.9%; p < 0.001). Compared with 2021 K-TIRADS4-1.0cm, 2021 K-TIRADS4-1.0~1.5cm showed higher specificity (44.9% vs. 47.9%; p = 0.018) without significant difference in other diagnostic measures. Compared with 2016 K-TIRADS, 2021 K-TIRADS (biopsy cut-offs, 0.5 cm for K-TIRADS 5; 1.0-1.5 cm for K-TIRADS 4) showed higher sensitivity (34.0% vs. 67.3%; p < 0.001) while maintaining specificity (89.4% vs. 88.2%; p = 0.790) in small nodules, and higher specificity (5.9% vs. 25.4%; p < 0.001) while maintaining sensitivity (100% vs. 98.7%; p = 0.132) in large nodules. CONCLUSIONS: In pediatric patients, 2021 K-TIRADS showed superior diagnostic accuracy to 2016 K-TIRADS, especially with a biopsy cut-off of 0.5 cm for K-TIRADS 5 and 1.0-1.5 cm for K-TIRADS 4. KEY POINTS: ⢠All simulated 2021 K-TIRADS showed higher accuracy than 2016 K-TIRADS. ⢠2021 K-TIRADS with cut-off size for K-TIRADS 5 of 0.5 cm showed lower specificity but higher sensitivity and accuracy than that of 1.0 cm. ⢠Compared with 2016 K-TIRADS, 2021 K-TIRADS (biopsy cut-offs, 0.5 cm for K-TIRADS 5; 1.0-1.5 cm for K-TIRADS 4) showed higher sensitivity while maintaining specificity in small nodules, and higher specificity while maintaining sensitivity in large nodules.
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Neoplasias da Glândula Tireoide , Nódulo da Glândula Tireoide , Humanos , Criança , Nódulo da Glândula Tireoide/diagnóstico por imagem , Estudos Retrospectivos , Neoplasias da Glândula Tireoide/patologia , Ultrassonografia/métodos , Medição de Risco/métodos , República da Coreia/epidemiologiaRESUMO
In medicine, confounding variables in a generalized linear model are often adjusted; however, these variables have not yet been exploited in a non-linear deep learning model. Sex plays important role in bone age estimation, and non-linear deep learning model reported their performances comparable to human experts. Therefore, we investigate the properties of using confounding variables in a non-linear deep learning model for bone age estimation in pediatric hand X-rays. The RSNA Pediatric Bone Age Challenge (2017) dataset is used to train deep learning models. The RSNA test dataset is used for internal validation, and 227 pediatric hand X-ray images with bone age, chronological age, and sex information from Asan Medical Center (AMC) for external validation. U-Net based autoencoder, U-Net multi-task learning (MTL), and auxiliary-accelerated MTL (AA-MTL) models are chosen. Bone age estimations adjusted by input, output prediction, and without adjusting the confounding variables are compared. Additionally, ablation studies for model size, auxiliary task hierarchy, and multiple tasks are conducted. Correlation and Bland-Altman plots between ground truth and model-predicted bone ages are evaluated. Averaged saliency maps based on image registration are superimposed on representative images according to puberty stage. In the RSNA test dataset, adjusting by input shows the best performances regardless of model size, with mean average errors (MAEs) of 5.740, 5.478, and 5.434 months for the U-Net backbone, U-Net MTL, and AA-MTL models, respectively. However, in the AMC dataset, the AA-MTL model that adjusts the confounding variable by prediction shows the best performance with an MAE of 8.190 months, whereas the other models show the best performances by adjusting the confounding variables by input. Ablation studies of task hierarchy reveal no significant differences in the results of the RSNA dataset. However, predicting the confounding variable in the second encoder layer and estimating bone age in the bottleneck layer shows the best performance in the AMC dataset. Ablations studies of multiple tasks reveal that leveraging confounding variables plays an important role regardless of multiple tasks. To estimate bone age in pediatric X-rays, the clinical setting and balance between model size, task hierarchy, and confounding adjustment method play important roles in performance and generalizability; therefore, proper adjusting methods of confounding variables to train deep learning-based models are required for improved models.
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Aprendizado Profundo , Radiologia , Humanos , Criança , Raios X , Fatores de Confusão Epidemiológicos , RadiografiaRESUMO
Background The validation of adult-based US risk stratification systems (RSSs) in the discrimination of malignant thyroid nodules in a pediatric population remains lacking. Purpose To estimate and compare the diagnostic performance of pediatric US RSSs based on five adult-based RSSs in the discrimination of malignant thyroid nodules in a pediatric sample. Materials and methods Pediatric patients (age ≤18 years) with histopathologically confirmed US-detected thyroid nodules at a tertiary referral hospital between January 2000 and April 2020 were analyzed retrospectively. The diagnostic performance of US-based fine-needle aspiration biopsy (FNAB) criteria in thyroid cancer detection was estimated. The following sensitivity analyses were performed: (a) scenario 1: nodules smaller than 1 cm, with the highest category additionally biopsied; (b) scenario 2, application of American College of Radiology Thyroid Imaging Reporting and Data System nodule size cutoffs to other RSSs; (c) scenario 3, scenarios 1 and 2 together. Generalized estimating equations (GEEs) were used for estimation. Results A total of 277 thyroid nodules in 221 pediatric patients (median age, 16 years [interquartile range {IQR}, 13-17]; 172 female; 152 of 277 patients [55%] malignant) were analyzed. The GEE-estimated sensitivity and specificity ranged from 70% to 78% (104 to 119 of 152 patients, based on each reader's interpretation) and from 42% to 78% (49 of 124 patients to 103 of 125 patients). In scenario 1, the missed malignancy rate was reduced from 32%-38% (41 of 134 patients to 34 of 83 patients) to 15%-21% (eight of 59 patients to 28 of 127 patients). In scenario 2, the unnecessary biopsy rate was reduced from 35%-39% (60 of 176 patients to 68 of 175 patients) to 20%-34% (18 of 109 patients to 62 of 179 patients). The highest accuracy was noted in scenario 3 (range, 71%-81%; 199 of 277 patients to 216 of 262 patients). Conclusion The diagnostic performances of the fine-needle aspiration biopsy criteria of five adult-based risk stratification systems were acceptable in the pediatric population and were improved by applying the American College of Radiology Thyroid Imaging Reporting and Data System size cutoff for nodules 1 cm or larger and allowing biopsy of the highest category nodules smaller than 1 cm. © RSNA, 2022 Online supplemental material is available for this article.
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Neoplasias da Glândula Tireoide , Nódulo da Glândula Tireoide , Adolescente , Adulto , Biópsia por Agulha Fina , Criança , Feminino , Humanos , Reprodutibilidade dos Testes , Estudos Retrospectivos , Medição de Risco , Neoplasias da Glândula Tireoide/diagnóstico por imagem , Neoplasias da Glândula Tireoide/patologia , Nódulo da Glândula Tireoide/diagnóstico por imagem , Nódulo da Glândula Tireoide/patologia , Ultrassonografia/métodosRESUMO
OBJECTIVE: To evaluate associations between postnatal imaging features and outcome of left-sided congenital diaphragmatic hernia, as defined by overall survival and a requirement for extracorporeal membrane oxygenation (ECMO). STUDY DESIGN: Newborns diagnosed prenatally with left-sided congenital diaphragmatic hernia between January 2013 and September 2021 were studied retrospectively. The esophageal deviation index was newly defined as the largest diameter from the midline to deviated gastric tube divided by the largest transverse diameter of the thoracic cavity on the radiograph. Regression analyses were performed to identify postnatal imaging features associated with overall survival and a requirement for ECMO. The predictive power (ie, area under the curve [AUC] of a time-dependent receiver operating characteristic curve) of prenatal, postnatal, and intraoperative findings for predicting survival were calculated. RESULTS: Ninety-seven patients (54 males; mean gestational age, 38.3 ± 1.9 weeks; mean birth weight, 2956.5 ± 540.0 g) were analyzed. The esophageal deviation index (adjusted hazard ratio [HR], moderate [≥0.19 to <0.24], 6.427 [P = .029]; severe [≥0.24], 33.007 [P < .001]) and right pneumothorax (adjusted HR, 8.763; P = .002) were associated with overall survival and with a requirement for ECMO. Liver herniation on postnatal ultrasound also was associated with overall survival (P < .001) and need for ECMO (P = .001). In addition, the AUC for prediction of 1-year survival from postnatal ultrasound was comparable with that of prenatally or intraoperatively detected liver herniation (0.93; 95% CI, 0.88-0.97). CONCLUSIONS: The esophageal deviation index, right pneumothorax, and liver herniation observed by postnatal imaging have prognostic value in patients with left-sided congenital diaphragmatic hernia.
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Hérnias Diafragmáticas Congênitas , Pneumotórax , Gravidez , Masculino , Feminino , Humanos , Recém-Nascido , Lactente , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Estudos Retrospectivos , Ultrassonografia Pré-Natal/métodos , Fígado/diagnóstico por imagemRESUMO
OBJECTIVE: To identify predictors of failed enema reduction in children with intussusception. METHODS: PubMed and EMBASE were searched for all studies published over a 20-year time frame, prior to March 25, 2020. Original articles that reported predictors of failed enema reduction were included. The pooled odds ratio (OR) for successful enema reduction according to various features was calculated. The combined estimates were meta-analytically pooled by random-effects modeling. The risk of bias was assessed using the National Institute of Health Quality Assessment Tool. This review was registered to the PROSPERO (CRD42020190178). RESULTS: A total of 38 studies, comprising 40,133 cases, were included. The shorter duration of symptoms (< 24 h; combined OR, 3.812; 95% CI, 2.150-6.759) and abdominal pain (combined OR, 2.098; 95% CI, 1.405-3.133) were associated with the success (all p < 0.001). Age < 1 year (combined OR, 0.385; 95% CI, 0.166-0.893; p = 0.026), fever (combined OR, 0.519; 95% CI, 0.371-0.725; p < 0.001), rectal bleeding (combined OR, 0.252; 95% CI, 0.165-0.387; p < 0.001), and vomiting (combined OR, 0.497; 95% CI, 0.372-0.664; p < 0.001) were associated with the failed reduction. The ascites (combined OR, 0.127; 95% CI, 0.044-0.368; p = 0.001), left-sided intussusception (combined OR, 0.121; 95% CI, 0.058-0.252; p < 0.001), and trapped fluid (combined OR, 0.179; 95% CI, 0.061-0.525; p = 0.017) on US were associated with the failed reduction. CONCLUSIONS: Successful predictors for intussusception reduction have been summarized. This evidence can help identify patients who are more likely to fail non-operative reduction and could be potential surgical candidates. KEY POINTS: ⢠A shorter duration of symptoms and presence of abdominal pain were associated with increased probability of success. ⢠Age (less than 1 year), presence of fever, rectal bleeding, vomiting, and presence of ascites, left-sided intussusception, or trapped fluid on ultrasonography were associated with decreased probability of success. ⢠This study suggests that various clinical and ultrasonography predictors would help identify patients who are more likely to fail nonoperative reduction and identify potential preoperative candidates.
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Intussuscepção , Criança , Enema , Humanos , Lactente , Intussuscepção/complicações , Intussuscepção/diagnóstico por imagem , Intussuscepção/terapia , Razão de Chances , Estudos Retrospectivos , UltrassonografiaRESUMO
OBJECTIVES: To evaluate the diagnostic performance of adult-based "American College of Radiology- Thyroid Imaging Reporting And Data System" (ACR-TIRADS) and "American Thyroid Association" (ATA) in the pediatric population. METHODS: MEDLINE/PubMed, EMBASE, Cochrane Library, and Web of Science databases were searched for articles investigating the diagnostic performance of each stratification system (ACR-TIRADS or ATA) and evaluated them according to three aspects: (a) the risk of malignancy in each category; (b) diagnostic performance using the classic indicators (sensitivity, specificity); and (c) diagnostic performance regarding fine needle aspiration/biopsy recommendation. In addition to pathologic diagnosis, we allowed imaging follow-up as the reference standard for benign nodules. RESULTS: Eight articles (1036 thyroid nodules) were included. For ACR-TIRADS, the pooled risk of malignancy in category was as follows: category 5 (59.3%); 4 (20.7%); 3 (11.0%); 2 (6.0%), and 1 (5.5%). For nodules of high suspicion of malignancy (category 4 or 5), the pooled sensitivity and specificity were 0.84 and 0.64. For ATA, the pooled risk of malignancy was as follows: category 5 (55.4%); 4 (34.2%); 3 (12.2%); and 2 (7.5%). For nodules of high suspicion of malignancy (category 4 or 5), the pooled sensitivity and specificity were 0.90 and 0.50. For category 5 nodules, the pooled specificity was significantly higher in ACR-TIRADS (p = 0.02). For ACR-TIRADS, the missed malignancy rate was 21.7% and the unnecessary biopsy rate was 62.7%. Information was not sufficient for this calculation with ATA. CONCLUSIONS: The diagnostic performance of ACR-TIRADS and ATA in the pediatric population was somewhat modest. Large studies are mandatory for further validation and future amendments. KEY POINTS: ⢠The pooled sensitivity and specificity for highly suspicious nodules (category 4 or 5) for ACR-TIRADS were 0.84 and 0.64, and for ATA were 0.90 and 0.50, respectively. ⢠When applying ACR-TIRADS for children, the pooled missed malignancy rate (21.7%) and unnecessary biopsy rates (62.7%) are still reasonably high. Insufficient information was available to perform these calculations for the ATA system. ⢠Current risk stratification systems, especially ACR-TIRADS, require modification by focusing more on increasing the sensitivity and decreasing the missed malignancy rate. Lowering size cut-off for biopsy would be a reasonable option.
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Neoplasias da Glândula Tireoide , Nódulo da Glândula Tireoide , Adulto , Biópsia por Agulha Fina , Criança , Humanos , Estudos Retrospectivos , Medição de Risco , Nódulo da Glândula Tireoide/diagnóstico por imagem , Ultrassonografia , Estados UnidosRESUMO
OBJECTIVES: To evaluate the outcome of staging chest CT and to identify clinicoradiological factors predictive of lung metastasis in patients with hepatoblastoma based on the 2017 PRE-Treatment EXTent of tumor (PRETEXT) system. METHODS: This bi-center study retrospectively identified patients diagnosed with hepatoblastoma between January 1998 and September 2019 in two tertiary hospitals. The primary outcome was the proportion of the patients who had lung metastasis at staging chest CT. The diagnostic accuracy of staging chest CT was calculated based on the 2017 PRETEXT criteria. The secondary outcome was the identification of factors predictive of lung metastasis using multivariable logistic regression. RESULTS: In total, 123 patients (median age, 1 year; interquartile range, 0-4 years; 59 female) were included. Among those, 28% (35/123; 95% confidence interval [CI], 21-37%) had lung metastasis at staging chest CT. The overall accuracy of staging chest CT was 96.8%. The proportion of lung metastasis in patients with stage I, II, III, and IV was 0%, 24% (12 of 49; 95% CI, 14-38%), 23% (9 of 40; 95% CI, 12-38%), and 56% (14 of 25; 95% CI, 37-73%), respectively. Multifocality (adjusted odds ratio, 6.7; 95% CI, 2.7-17.5; p < .001) and male sex (adjusted odds ratio, 3.1; 95% CI, 1.2-8.6; p = .02) were associated with the presence of lung metastasis. CONCLUSIONS: Twenty-eight percent of the patients with hepatoblastoma had lung metastasis at staging chest CT. Multifocality and male sex were predictive factors for lung metastasis on staging chest CT. KEY POINTS: ⢠The proportion of lung metastasis in patients with hepatoblastoma was 28%. ⢠The overall accuracy of staging chest CT was 97% based on the 2017 PRETEXT system. ⢠Hepatic tumor multifocality and male sex were predictors of lung metastasis.
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Hepatoblastoma , Neoplasias Hepáticas , Neoplasias Pulmonares , Criança , Feminino , Hepatoblastoma/diagnóstico por imagem , Hepatoblastoma/patologia , Humanos , Lactente , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/patologia , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Ambroxol (ABX) has been suggested as an augmentative pharmacological agent for neuronopathic Gaucher disease (nGD). This study assessed the long-term safety and efficacy of combined therapy with high-dose ABX and enzyme replacement therapy (ERT) in nGD. METHODS: ABX+ERT therapy was administered for 4.5 years in four patients with nGD. ABX was initiated at a dose of 1.5 mg/kg/day, and the dose was escalated up to 27 mg/kg/day. The target plasma level was 10 µmol/L or less. The changes in glucocerebrosidase activity, biochemical, safety and neurocognitive findings were assessed. RESULTS: Enhanced residual GCcase activity was observed in all patients, as evidenced in both in vitro and in vivo studies. During the first 2 years of study with ABX (up to 21 mg/kg/day), mean seizure frequencies and neurocognitive function worsened. After ABX dosage was increased up to 27 mg/kg/day of ABX, its trough plasma concentration was 3.2-8.8 µmol/L. Drug-to-drug interaction, especially with antiepileptic drug significantly affected the pharmacokinetic parameters of ABX. Importantly, at 27 mg/kg/day of ABX, the seizure frequencies markedly decreased from the baseline, and the neurocognitive function was improved. In addition, Lyso-Gb1, a biomarker for the severity and progression of GD, was normalised in all patients. High-dose ABX was well-tolerated with no severe adverse events. CONCLUSIONS: Long-term treatment with high-dose ABX+ERT was safe and might help to arrest the progression of the neurological manifestations in GD.
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Ambroxol/administração & dosagem , Terapia de Reposição de Enzimas , Epilepsias Mioclônicas/tratamento farmacológico , Doença de Gaucher/tratamento farmacológico , Adolescente , Biomarcadores/sangue , Criança , Relação Dose-Resposta a Droga , Epilepsias Mioclônicas/sangue , Epilepsias Mioclônicas/patologia , Feminino , Doença de Gaucher/sangue , Doença de Gaucher/patologia , Glucosilceramidase/sangue , Humanos , MasculinoRESUMO
BACKGROUND: Respiratory artifacts impair image quality of magnetic resonance imaging (MRI) in children who cannot hold breath during MRI examination. PURPOSE: To compare the quality of free-breathing contrast-enhanced 3D T1-weighted (T1W) images of the upper abdomen in children using Cartesian acquisition (Cartesian eTHRIVE), stack-of-stars acquisition with spectral fat suppression (3D VANE eTHRIVE), and stack-of-stars acquisition with fat suppression using modified Dixon (3D VANE mDixon). MATERIAL AND METHODS: Pediatric patients (aged <19 years) who underwent whole-body MRI with free-breathing contrast-enhanced T1W axial scans of upper abdomen using Cartesian eTHRIVE, 3D VANE eTHRIVE, and 3D VANE mDixon were enrolled. Image quality parameters were assessed including overall image quality, hepatic edge sharpness, hepatic vessel clarity, respiratory artifacts, radial artifacts, lesion conspicuity, and lesion edge sharpness using the Likert scale, where a lower score indicated poorer image quality. The coefficients of variation of signal intensity of liver and spleen were analyzed. RESULTS: In 41 patients, 3D VANE eTHRIVE showed the highest scores for all image quality parameters (P ≤ 0.001). 3D VANE eTHRIVE also showed higher scores for respiratory (P ≤ 0.001) and radial artefacts than 3D VANE mDixon (P = 0.001). There were no significant differences in coefficients of variation of signal intensity of the liver and spleen between 3D VANE eTHRIVE and 3D VANE mDixon. Acquisition time was longer for 3D VANE eTHRIVE (81.26 ± 16 s) than for Cartesian eTHRIVE (7.87 ± 0.95 s) and 3D VANE mDixon (76.66 ± 12.4 s, P < 0.001). CONCLUSION: The application of stack-of-stars acquisition to 3D T1W abdominal MRI resulted in better image quality than Cartesian acquisition in free-breathing children. In stack-of-stars acquisition, spectral fat suppression resulted in better image quality and fewer artifacts than mDixon.
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Abdome/diagnóstico por imagem , Meios de Contraste , Imageamento Tridimensional , Imageamento por Ressonância Magnética/métodos , Gordura Abdominal , Artefatos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Respiração , Estudos RetrospectivosRESUMO
BACKGROUND: Validated non-invasive examinations are necessary to monitor liver fibrosis in children with biliary atresia (BA) after the Kasai procedure. PURPOSE: To evaluate the diagnostic accuracy of two-dimensional shear wave elastography (2D-SWE), transient elastography (TE), and the serologic biomarkers of aspartate transaminase-to-platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score for evaluating native liver fibrosis in children with BA. MATERIAL AND METHODS: We retrospectively reviewed same-day 2D-SWE and TE liver stiffness (LS) measurements of 63 patients with BA who underwent the Kasai procedure. The APRI and FIB-4 score were computed. Hepatic fibrosis was categorized into three clinical categories based on the ultrasound (US) hepatic morphology and clinical manifestations of liver cirrhosis: I, pre-cirrhotic liver state (n = 15); II, US and/or clinical signs of liver cirrhosis with compensated liver function (n = 27); and III, liver cirrhosis with decompensated liver function (n = 21). We compared area under the receiver operating characteristic curve (AUC) data among 2D-SWE, TE, APRI, and FIB-4 score. Combined evaluation of serologic fibrosis indices and US elastography was conducted and AUCs of combinations were analyzed. RESULTS: 2D-SWE, TE, APRI, and FIB-4 score showed good to excellent diagnostic accuracy for differentiating clinical categories (AUCs 0.779-0.955). AUC values were significantly increased after adding TE to FIB-4 score for detecting liver cirrhosis (P = 0.02). CONCLUSION: 2D-SWE, TE, APRI, and FIB-4 score are accurate non-invasive markers for monitoring native liver fibrosis in patients with BA. Combined use of serologic markers and US elastography could yield more accurate diagnoses of liver fibrosis than serologic markers alone.
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Atresia Biliar/cirurgia , Técnicas de Imagem por Elasticidade/métodos , Cirrose Hepática/diagnóstico , Portoenterostomia Hepática/efeitos adversos , Ultrassonografia/métodos , Adolescente , Aspartato Aminotransferases/sangue , Biomarcadores/sangue , Criança , Pré-Escolar , Humanos , Lactente , Cirrose Hepática/sangue , Cirrose Hepática/diagnóstico por imagem , Cirrose Hepática/etiologia , Contagem de Plaquetas , Portoenterostomia Hepática/métodos , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade , Adulto JovemRESUMO
OBJECTIVES: To estimate the technical performance of acoustic radiation force impulse (ARFI) imaging (two-dimensional shear wave elastography [2D-SWE] and point shear wave elastography [p-SWE]) for measuring renal parenchymal stiffness. METHODS: EMBASE and PubMed databases were searched for studies reporting technical performance of ARFI imaging in terms of technical failure, interobserver agreement, and/or intraobserver agreement. The proportion of technical failure and intraclass correlation coefficients (ICCs) for interobserver and intraobserver agreement was pooled. The pooled estimates of native and transplanted kidneys were obtained separately. Meta-regression and subgroup analyses were conducted to explore heterogeneity. RESULTS: Twenty-four studies (2993 patients) were included. The pooled proportions of technical failure were 4.3% (95% confidence interval [CI] 2.2-8.5%) and 6.6% (95% CI 4.0-10.7%) in native and transplanted kidneys, respectively. The pooled ICCs of interobserver agreement were 0.70 (95% CI 0.68-0.83) and 0.81 (95% CI 0.68-0.89), indicating moderate and good agreement in native and transplanted kidneys, respectively. The pooled ICC showed good (0.77; 95% CI 0.49-0.91) intraobserver agreement in native kidneys. Regarding interobserver agreement in transplanted kidneys, ROI location (mid pole only versus others) was a significant factor of heterogeneity (P = .04). CONCLUSIONS: The ARFI-based SWE techniques show good technical performance for measuring renal parenchymal stiffness. The wide range of SWE protocols necessitates development of standardized guidelines on the use of renal ARFI imaging.
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Técnicas de Imagem por Elasticidade , Bases de Dados Factuais , Humanos , Rim/diagnóstico por imagem , Reprodutibilidade dos TestesRESUMO
Neurofibromatosis type 1 (NF1) is caused by heterozygous mutation in the NF1 gene. NF1 is one of the most common human genetic diseases. However, the overall genotype-phenotype correlation has not been known, due to a wide spectrum of genotypic and phenotypic heterogeneity. Here we describe the detailed clinical and genetic features of 427 Korean NF1 patients from 389 unrelated families. Long range PCR and sequencing of genomic DNA with multiplex ligation-dependent probe amplification analysis identified 250 different NF1 mutations in 363 families (93%), including 94 novel mutations. With an emphasis on phenotypes requiring medical attention (classified and termed: NF1+), we investigated the correlation of NF1+ and mutation types. NF1+ was more prevalent in patients with truncating/splicing mutations and large deletions than in those with missense mutations (59.6%, 64.3% vs. 36.6%, p = 0.001). This difference was especially significant in the patients younger than age 19 years. The number of items in NF1+ was a higher in the former groups (0.95 ± 0.06, 1.18 ± 0.20 vs. 0.56 ± 0.10, p = 0.002). These results suggest that mutation types are associated not only with higher prevalence of severe phenotypes in NF1 but also with their earlier onset.
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Estudos de Associação Genética , Neurofibromatose 1/genética , Neurofibromina 1/genética , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Estudos de Coortes , Análise Mutacional de DNA , Feminino , Genótipo , Heterozigoto , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase Multiplex , Mutação , Fenótipo , Adulto JovemRESUMO
BACKGROUND: Magnetic resonance elastography (MRE) using a gradient-recalled echo (GRE) or a recently available spin-echo echo-planar imaging (SE-EPI) sequence is a promising noninvasive method for measuring liver stiffness. However, it sometimes fails to measure stiffness values, thereby resulting in technical failures. PURPOSE: To assess and compare technical failures of MRE for measuring liver stiffness between GRE and SE-EPI sequences. STUDY TYPE: Systematic review and meta-analysis. POPULATION: Eight studies with both GRE and SE-EPI, 22 studies with only GRE, one study with only SE-EPI. FIELD STRENGTH/SEQUENCE: Either 1.5 or 3T MRE using GRE and/or SE-EPI. ASSESSMENT: Through an Ovid-MEDLINE and EMBASE database search, original articles investigating the proportion of MRE technical failures in the measurement of liver stiffness published up until October 2018 were screened and selected. STATISTICAL ANALYSIS: The pooled proportions of technical failures under GRE and SE-EPI were calculated using random-effects meta-analysis of single proportions and inverse variance for calculating weights. Subgroup analyses were performed to explore the covariates affecting heterogeneity. Head-to-head comparisons of technical failure between the sequences were conducted with eight MRE studies using both GRE and SE-EPI. RESULTS: The pooled proportion of technical failure under GRE MRE was 5.8% (95% confidence interval [CI], 4.6-7.4%), and a subgroup analysis showed higher technical failure rates at 3T than at 1.5T. The pooled proportion of technical failure under SE-EPI MRE was 2.0% (95% CI, 1.3-3.4%), without significant differences (P = 0.38-0.89) being observed in the subgroup analyses. In the eight studies comparing the two sequences, failure was more frequently observed with GRE than with SE-EPI (9.4% vs. 1.9%; P < 0.01). DATA CONCLUSION: MRE conducted with SE-EPI sequences showed a lower technical failure rate than GRE sequences. With GRE sequences, a magnetic field of 3T was associated with higher technical failure rates than was 1.5T. LEVEL OF EVIDENCE: 1 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2020;51:1086-1102.
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Técnicas de Imagem por Elasticidade , Imagem Ecoplanar , Humanos , Cirrose Hepática , Imageamento por Ressonância Magnética , Reprodutibilidade dos TestesRESUMO
OBJECTIVES: To compare the feasibility and clinical applicability of ElastQ imaging (Philips Healthcare, Best, the Netherlands) with that of SuperSonic shear imaging (SSI; SuperSonic Imagine, Aix-en-Provence, France) using an elastographic phantom and a pilot study of patients. METHODS: Two-dimensional shear wave elastography measurement was performed by ElastQ imaging and SSI by 2 radiologists. An elastographic phantom with 5 target elasticities at 2 acquisition depths was used. The coefficients of variation and intraclass correlation coefficients (ICCs) were evaluated for repeatability and interobserver agreement, respectively. The mean elasticities of the systems at each target were compared. The proportions of measurements that were out of the range of expected values and measurement errors were calculated to determine accuracy. Liver stiffness (LS) was measured by both systems in 27 children and young adult patients with various liver diseases. RESULTS: Both systems provided high repeatability in elasticity measurements of phantom targets (coefficients of variation, 0.69%-15.82%), and there was excellent interobserver agreement (ICC, 0.992). Most (90%) mean elasticities of targets were significantly different between the techniques (P ≤ .002) and acquisition depths (P ≤ .004). ElastQ imaging had significantly lower proportions of out-of-range measurements and measurement errors (P ≤ .003) than SSI. In patients with liver disease, LS measurements of the systems were strongly correlated (ρ = 0.955; P < .001) and had excellent agreement (ICC, 0.951; P < .001). CONCLUSIONS: ElastQ imaging had comparably good results in terms of repeatability, interobserver agreement, and accuracy in the phantom model compared with SSI. The pilot patient study showed strong correlations in LS values between the systems.
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Técnicas de Imagem por Elasticidade/métodos , Hepatopatias/diagnóstico por imagem , Criança , Módulo de Elasticidade , Feminino , Humanos , Masculino , Imagens de Fantasmas , Projetos Piloto , Estudos RetrospectivosRESUMO
OBJECTIVES: To investigate the diagnostic value of transient elastography (TE), 2-dimensional (2D) shear wave elastography (SWE), and the serologic fibrosis indices aspartate transaminase-to-platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score for Wilson disease (WD). METHODS: We retrospectively identified patients with a diagnosis of WD who underwent TE and 2D SWE on the same day. Their APRI and FIB-4 scores were calculated. Hepatic involvement was classified into 5 clinical categories (I-V) based on the laboratory findings, hepatic morphologic characteristics on ultrasound (US) imaging, and clinical symptoms of cirrhosis: I, normal (n = 17); II, only biochemical abnormality (n = 15); III, altered hepatic morphologic characteristics (n = 10); IV, compensated liver cirrhosis (n = 3); and V, decompensated liver cirrhosis (n = 0). We compared the area under the receiver operating characteristic curve (AUROC) data for TE, 2D SWE, the APRI, and the FIB-4 score. A combined assessment of the serologic markers and US elastography was performed, and the AUROCs of the combinations were compared. RESULTS: Forty-five patients were included in the study (median age, 16.0 years; range, 3-35 years). Transient elastography, 2D SWE, and APRI were comparable in distinguishing the clinical categories (AUROC, 0.799-0.928). The FIB-4 score showed lower diagnostic value in distinguishing clinical category I from the other categories (AUROC, 0.647). Combining the serologic markers and US elastography significantly increased the AUROC value of the FIB-4 score (with TE and 2D SWE, P = .01 and .02). CONCLUSIONS: Transient elastography and 2D SWE showed excellent diagnostic accuracy for differentiating the clinical categories of hepatic involvement. The APRI showed better diagnostic performance than the FIB-4 score. The assessment of hepatic manifestations in WD can be improved by combining US elastography with serologic indices.
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Técnicas de Imagem por Elasticidade , Degeneração Hepatolenticular , Adolescente , Adulto , Aspartato Aminotransferases , Criança , Pré-Escolar , Degeneração Hepatolenticular/diagnóstico por imagem , Degeneração Hepatolenticular/patologia , Humanos , Cirrose Hepática , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVE: To assess the technical performance (proportion of technical failure and unreliable measurements) of shear wave elastography (SWE) for assessing liver stiffness in pediatric and adolescent patients. METHODS: We searched Ovid-MEDLINE and EMBASE databases for eligible studies and selected original articles investigating transient elastography (TE), point shear wave elastography (pSWE), or two-dimensional SWE (2D-SWE) for measuring liver stiffness in pediatric and adolescent patients. A quantitative synthesis of studies reporting technical failures and/or unreliable measurements of TE, pSWE, or 2D-SWE is presented. Meta-analytic pooling was conducted using the random effects model. Meta-regression analysis was conducted to explore potential causes of heterogeneity. RESULTS: Forty of 69 studies (58%) provided technical performance information. Technical failure data were reported in 3 TE, 6 pSWE, and 8 2D-SWE studies. Unreliable measurement data were provided in 21 TE, 4 pSWE, and 1 2D-SWE study. The pooled proportion of unreliable measurements of TE was 12.1%. Meta-regression analysis showed that the study population size and readers' blinding to pathologic results affected the study's heterogeneity. The pooled proportions of technical failure during pSWE and 2D-SWE were 4.1% and 2.2%, respectively, demonstrating no significant difference between the techniques. CONCLUSIONS: We reviewed the technical performance of SWE, especially the rate of unreliable measurements from TE studies and rates of technical failure from pSWE and 2D-SWE studies. Considering the importance of technical performance for clinical validation of SWE, numbers of and reasons for technical failure and unreliable measurements should be reported in future studies. Further efforts are necessary to standardize SWE reliability criteria. KEY POINTS: ⢠Most TE studies reported rate of unreliable measurements, whereas pSWE and 2D-SWE studies were likely to report rates of technical failure. ⢠The pooled proportion of unreliable measurements of TE was 12.1%. ⢠The pooled proportions of technical failure during pSWE and 2D-SWE were 4.1% and 2.2%, respectively, demonstrating no significant difference between the techniques.
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Técnicas de Imagem por Elasticidade/métodos , Cirrose Hepática/diagnóstico , Fígado/diagnóstico por imagem , Adolescente , Criança , Elasticidade , Humanos , Fígado/fisiopatologia , Cirrose Hepática/patologia , Reprodutibilidade dos TestesRESUMO
OBJECTIVES: To assess the diagnostic performance of ultrasound (US) elastography in evaluating portal hypertension in children and compare the liver and spleen stiffness values between the portal hypertension and control groups. METHODS: Studies in the MEDLINE and Embase databases were selected that investigated the diagnostic performance of US elastography in children with portal hypertension up to December 21, 2017. Pooled sensitivity and specificity data were assessed by hierarchical logistic regression modeling. RESULTS: Eleven studies were included in the systematic review, and a meta-analysis could be conducted in 7 of these publications to evaluate the diagnostic performance of US elastography. The summary sensitivity and specificity of this method for liver stiffness were 90% (95% confidence interval [CI], 83%-94%) and 79% (95% CI, 73%-84%), respectively, and the area under the hierarchical summary receiver operating characteristic curve was 0.92 (95% CI, 0.90-0.94). A subgroup analysis of 5 transient elastographic studies revealed similar diagnostic performance (sensitivity, 90%; specificity, 78%). In 10 of the 11 studies that investigated liver stiffness and 2 of the 3 studies that also measured spleen stiffness, patients in the portal hypertension group had a significantly higher stiffness value than the control group (P < .05). CONCLUSIONS: Ultrasound elastography shows good performance in diagnosing portal hypertension and can identify significant differences in liver and spleen stiffness in children with this condition. This method thus has considerable potential as a noninvasive tool for screening portal hypertension-related complications in children with chronic liver disease.
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Técnicas de Imagem por Elasticidade/métodos , Hipertensão Portal/diagnóstico por imagem , Fígado/diagnóstico por imagem , Fígado/patologia , Baço/diagnóstico por imagem , Baço/patologia , Adolescente , Adulto , Criança , Pré-Escolar , Bases de Dados Factuais , Humanos , Hipertensão Portal/patologia , Lactente , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Adulto JovemRESUMO
Purpose To develop and validate a scoring system based on clinical and imaging features to predict the risk for biliary atresia in patients with neonatal cholestasis. Materials and Methods Patients with neonatal cholestasis who underwent both ultrasonography (US) and hepatobiliary scintigraphy (n = 480) were retrospectively identified from two tertiary referral hospitals from January 2000 to February 2017. Patients from one hospital were classified as the derivation cohort (n = 371), and those from the other hospital were classified as the validation cohort (n = 109). Clinical and imaging features associated with biliary atresia were assessed. Histopathologic or intraoperative cholangiographic findings served as the reference standard for biliary atresia. A prediction model was developed by using logistic regression and was then transformed into a scoring system. The scoring system was internally and externally validated. Results Among the 371 patients in the derivation cohort, 97 (26.15%) had biliary atresia. A scoring system was constructed with the following variables: full-term birth, presence of the triangular cord sign at US, abnormal gallbladder morphology at US, and failure of radioisotope excretion to the small bowel at hepatobiliary scintigraphy. The maximum possible score with this system is 7 points. This system enabled differentiation of biliary atresia in the derivation cohort (C statistic, 0.981; 95% confidence interval [CI]: 0.970, 0.992) and the validation cohort (C statistic, 0.995; 95% CI: 0.987, 1.000). The risk score also showed good calibration in both the derivation and the validation cohorts (P = .328 and P = .281, respectively). Conclusion A simple scoring system combining clinical and imaging features can help accurately estimate the risk for biliary atresia in patients with neonatal cholestasis.