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Abstract Background Chronic enteropathy associated with SLCO2A1 gene (CEAS) is a unique type of inflammatory bowel disease. CEAS is monogenic disease and is thought to develop from childhood, but studies on pediatric CEAS are scarce. We analyzed characteristics of pediatric CEAS. Methods Eleven patients diagnosed with CEAS at Seoul National University Children’s Hospital were identified and analyzed. Clinical data of patients were collected. Sanger sequencing of SLCO2A1 was performed on all patients. Results Patients were diagnosed at a median age of 16.0 years (IQR 11.0 ~ 20.0), and the median age at symptoms onset was only 4.0 years (IQR 2.5 ~ 6.0). Growth delay was observed at the time of diagnosis. Patients showed multiple ulcers or strictures in the small intestine, while the esophagus and colon were unaffected in any patients. Almost half of the patients underwent small intestine resection. The major laboratory features of pediatric CEAS include iron deficiency anemia (IDA), hypoalbuminemia, and near-normal levels of C-reactive protein (CRP). Two novel mutations of SLCO2A1 were identified. The most prevalent symptoms were abdominal pain and pale face. None of the immunomodulatory drugs showed a significant effect on CEAS. Conclusions Pediatric CEAS typically develop from very young age, suggesting it as one type of monogenic very early onset inflammatory bowel disease. CEAS can cause growth delay in children but there is no effective treatment currently. We recommend screening for SLCO2A1 mutations to pediatric patients with chronic IDA from a young age and small intestine ulcers without elevation of CRP levels.

Developmental dysplasia of the hip is a condition characterized by hip joint instability due to acetabular dysplasia in infancy, necessitating precise ultrasound examination. Legg- Calvé-Perthes disease is caused by a temporary disruption in blood flow to the femoral head during childhood, progressing through avascular, fragmentation, re-ossification, and residual stages. Slipped capital femoral epiphysis is a condition where the femoral head shifts medially along the epiphyseal line during adolescence due to stress, such as weight-bearing. Differentiating between transient hip synovitis and septic arthritis may require joint fluid aspiration. Osteomyelitis can be associated with soft tissue edema and osteolysis. When multiple lesions are present, it is essential to distinguish between Langerhans cell histiocytosis and metastatic neuroblastoma. This review will introduce imaging techniques and typical findings for these conditions.

Purpose This study explored the feasibility of utilizing a deep learning artificial intelligence (AI) model to detect ileocolic intussusception on grayscale ultrasound images. Methods This retrospective observational study incorporated ultrasound images of children who underwent emergency ultrasonography for suspected ileocolic intussusception. After excluding video clips, Doppler images, and annotated images, 40,765 images from two tertiary hospitals were included (positive-to-negative ratio: hospital A, 2,775:35,373; hospital B, 140:2,477). Images from hospital A were split into a training set, a tuning set, and an internal test set (ITS) at a ratio of 7:1.5:1.5. Images from hospital B comprised an external test set (ETS). For each image indicating intussusception, two radiologists provided a bounding box as the ground-truth label. If intussusception was suspected in the input image, the model generated a bounding box with a confidence score (0-1) at the estimated lesion location. Average precision (AP) was used to evaluate overall model performance. The performance of practical thresholds for the model-generated confidence score, as determined from the ITS, was verified using the ETS. Results The AP values for the ITS and ETS were 0.952 and 0.936, respectively. Two confidence thresholds, CTopt and CTprecision, were set at 0.557 and 0.790, respectively. For the ETS, the perimage precision and recall were 95.7% and 80.0% with CTopt, and 98.4% and 44.3% with CTprecision. For per-patient diagnosis, the sensitivity and specificity were 100.0% and 97.1% with CTopt, and 100.0% and 99.0% with CTprecision. The average number of false positives per patient was 0.04 with CTopt and 0.01 for CTprecision. Conclusion The feasibility of using an AI model to diagnose ileocolic intussusception on ultrasonography was demonstrated. However, further study involving bias-free data is warranted for robust clinical validation.

Objective: Adequate iodine intake is essential for growing children, and thyroid volume (Tvol) is considered as an indicator of iodine status. We investigated Tvol and goiter using ultrasonography (US) and their association with iodine status in 228 6-year-old children living in Korea. Methods: Iodine status was assessed using urine iodine concentration (UIC) and categorized as deficient (

Abstract Background Abdominal pain, which is a common cause of children presenting to the paediatric emergency department (PED), is often evaluated by ultrasonography (US). However, uncertainty in US reports may necessitate additional imaging. Objective In this study, we evaluated factors contributing to uncertainty in paediatric abdominal US reports in the PED. Materials and methods This retrospective cohort study included children younger than 18 years of age who underwent abdominal US in the PED of the study hospital between January 2017 and December 2019. After exclusion, the researchers manually reviewed and classified all US reports as ‘certain’ or ‘uncertain’. Univariate and multivariate logistic regression analyses were performed to identify the factors contributing to uncertain reports. Results In total, 1006 patients were included in the final analysis., 796 patients were tagged as having certain reports, and 210 as having uncertain reports. Children with uncertain reports had a significantly higher rate of undergoing an additional computed tomography (CT) scan (31.0% vs. 2.5%, p

Abstract Background To describe the radiological features of Gorham–Stout disease (GSD) as evaluated using plain radiography and dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) imaging techniques. Methods Clinical and conventional imaging data were retrospectively reviewed for 15 patients with GSD between January 2001 and December 2020. After December 2018, DCMRL examinations were performed for lymphatic vessel evaluation in patients with GSD and reviewed in four patients. Results The median age at diagnosis was 9 years (range: 2 months–53 years). The clinical manifestations were dyspnea in seven patients (46.7%), sepsis in 12 (80.0%), orthopedic problems in seven (46.7%), and bloody chylothorax in seven (46.7%). The common sites of osseous involvement were the spine (73.3%) and pelvic bone (60.0%). Among the non-osseous involvements, peri-osseous infiltrative soft-tissue abnormalities adjacent to the area of bone involvement were the most common (86.7%), followed by splenic cysts (26.7%) and interstitial thickening (26.7%). DCMRL demonstrated weak central conducting lymphatic flow in two patients with abnormal giant tortuous thoracic ducts and no flow in one patient. All patients who underwent DCMRL in this study presented with altered anatomical lymphatics and functional flow with collateralization. Conclusion DCMRL imaging and plain radiography are very useful for determining the extent of GSD. DCMRL is a novel imaging tool for the visualization of abnormal lymphatics in patients with GSD, which helps in further treatment. Therefore, in patients with GSD, it might be necessary to obtain not only plain radiographs but also MR and DCMRL images.

Purpose This study aimed to evaluate the usefulness of two-dimensional shear wave elastography (2D-SWE) in diagnosing hepatic veno-occlusive disease (VOD) in pediatric patients. Methods This study retrospectively included pediatric patients who underwent hematopoietic stem cell transplantation (HSCT) between November 2019 and January 2021. All 34 patients (8.7±5.0 years) were examined using 2D-SWE for an initial diagnosis. A subgroup analysis was performed using the data from follow-up examinations of patients diagnosed with VOD. The characteristics of the initial VOD diagnosis were compared with the longitudinal changes observed in VOD patients who underwent multiple ultrasound examinations. Results In total, 19 patients were diagnosed with VOD at 17.6±9.4 days after HSCT. All VOD patients showed hepatomegaly, ascites, and gallbladder wall thickening. Liver stiffness was higher in VOD patients than in non-VOD patients (12.4±1.1 vs. 6.3±0.8 kPa, P

BackgroundWe evaluated the long-term clinical outcomes of nephrocalcinosis (NC) according to etiology and grade in preschool-age children with NC.MethodsWe retrospectively analyzed the clinical outcomes and disease grade of children with NC classified into three groups according to etiology: prematurity, tubular disorders, and others.ResultsOverall, 67 children were diagnosed with NC [median age, 0.76 years; interquartile range (IQR) 0.46–2.14 years]. The etiologies of NC included prematurity (28.4%), tubular disorders (25.4%), and others (46.3%). Moreover, 56 (83.6%) children were asymptomatic and diagnosed accidentally through kidney ultrasonography. Newly diagnosed underlying diseases were greater in the tubular disorders group than in the other two groups (P = 0.001). Significantly more newly diagnosed NCs were grade 3 than grade 1 (P = 0.003). The median estimated glomerular filtration rate (eGFR) changed from 96.1 (IQR 68.8–119.2) ml/min/1.72 m2 at diagnosis to 90.9 (IQR 76.4–106.4) ml/min/1.72 m2 at the last follow-up, without a significant difference (P = 0.096). Changes in the kidney function did not differ according to etiology. However, patients without improvement in NC grade showed a decrease in eGFR from 98.1 (IQR 71.1–132.9) to 87.4 (IQR 74.0–104.1) ml/min/1.73 m2 (P = 0.023), while patients with improved NC grade did not show any change in the kidney function.ConclusionsEarly recognition, especially in NC grade 3, can help uncover further diagnoses, such as tubular disorders. Long-term kidney function depends on whether the NC grade improves.

Jaundice in children have various etiologies. Among them, physiological jaundice is a very common disease observed in more than half of full-term neonates. When jaundice persists or develops after 2 weeks of age, the total/direct bilirubin is measured in consideration of the possibility of cholestasis. In case of cholestasis, imaging studies differentiate biliary atresia and other disorders of the extrahepatic bile ducts. In this review, we briefly presented the major differential diseases of cholestasis in children and introduced diagnostic imaging techniques, including normal findings.

Hypoxic-ischemic encephalopathy in neonates is an important cause of brain damage that leads to severe neurological sequelae or death. Brain injury patterns on magnetic resonance imaging (MRI) scans are used to predict neurodevelopmental outcome severity. This case series describes the clinical manifestations and neurologic outcomes of four preterm infants with isolated deep gray matter injuries. Basal ganglia and thalamic lesions were noted without white matter and cerebral cortex lesion on brain MRI. All patients were preterm infants born at less than 33 weeks’ gestation and required resuscitation in the delivery room. All had seizures during the neonatal period requiring anti-seizure medications. Severe neurologic disability was identified in three patients using neurodevelopmental assessment tools. Another patient has not been evaluated with assessment tools yet as he was 2 months’ corrected age, but he was supported by home ventilation via a tracheostomy due to insufficient self-respiration. This case series demonstrates that isolated deep gray matter injuries in preterm infants could predict severe neurodevelopmental outcomes.

Purpose This study aimed to assess the feasibility of shear-wave elastography (SWE) for assessing the testicular involvement of hematologic malignancies in children and young adults. Methods Eight patients (mean age, 11.0 years; range, 0.8 to 20 years) with biopsy-confirmed testicular involvement of hematologic malignancy between January 2018 and December 2020 were retrospectively evaluated. Multiparametric ultrasound examinations, including grayscale, color Doppler ultrasonography (CDUS), and SWE, were performed. Stiffness was measured in the involved testicular area and contralateral normal parenchyma. If there was bilateral testicular involvement, the stiffness of the involved area and the adjacent normal echoic parenchyma was measured on one testis. The Mann-Whitney U test was used to compare stiffness values. Results On grayscale, the testicular lesions were noted as a solitary mass in one patient, multiple lesions in four patients, and diffuse involvement in three patients. On CDUS and SWE, all patients demonstrated increased vascularity, and the stiffness of the involved area was higher than the values of normal parenchyma (the involved area vs. normal parenchyma, 11.6 kPa [3.9-20.2 kPa] vs. 2.9 kPa [1.1-3.7 kPa], P=0.003). The ratio of stiffness between the involved area and normal parenchyma was 3.4, ranging from 1.9 to 5.1. One patient showed decreased stiffness on follow-up SWE after treatment (affected testis vs. normal testis: initial, 13.8 vs. 3.2 kPa; 1 year later, 2.2 vs. 2.4 kPa). Conclusion Increased testicular stiffness on SWE in children and young adults with hematologic malignancies suggests the possibility of testicular involvement.

The Korean Society of Radiology and Medical Guidelines Committee amended the existing 2016 guidelines to publish the “Korean Clinical Practice Guidelines for Adverse Reactions to Iodide Contrast for Injection and Gadolinium Contrast for MRI: The Revised Clinical Consensus and Recommendations (2022 Third Edition).” Expert members recommended and approved by the Korean Society of Radiology, the Korean Academy of Asthma, Allergy and Clinical Immunology, and the Korean Nephrology Society participated together. According to the expert consensus or systematic literature review, the description of the autoinjector and connection line for the infection control while using contrast medium, the acute adverse reaction, and renal toxicity to iodized contrast medium were modified and added. We would like to introduce the revised contents.

Purpose The purpose of this study was to use shear-wave elastography (SWE) to assess testicular stiffness changes during the Valsalva maneuver in adolescents and young adults with varicocele, to compare these changes according to clinical severity, and to evaluate the role of SWE in the diagnosis of varicocele. Methods This study included patients undergoing testicular ultrasonography for the diagnosis of varicocele or for post-varicocelectomy follow-up between June 2016 and February 2017. Fifty-four testicles of 27 consecutive patients (mean age, 15.9 years) were classified by clinical grade (grade 0-3). Using SWE, mean testicular stiffness (Emean) was measured at rest and during the Valsalva maneuver. The correlations between multiple ultrasonographic parameters (volume asymmetry, Emean at rest, and absolute and percentage changes in Emean during the Valsalva maneuver) and clinical grade were assessed using the Spearman correlation test. Results The Emean at rest was similar across clinical grades. During the Valsalva maneuver, increased testicular stiffness was frequently observed in patients with grade 2 or 3 varicocele but rarely observed in those with grade 0 or 1 varicocele. The changes in Emean were positively correlated with the clinical grade (all P0.05). Conclusion A transient, reversible increase in testicular stiffness during the Valsalva maneuver was observed in adolescents with high-grade varicocele, and the degree of stiffness change was correlated with the clinical grade. Stiffness change identified using SWE during the Valsalva maneuver is a potential surrogate indicator of venous congestion.

PurposeThis study aimed to assess the feasibility of contrast-enhanced ultrasound (CEUS) for the diagnosis of acute pyelonephritis (APN) in pediatric patients with febrile urinary tract infection (UTI).Materials and methodsBetween March 2019 and January 2021, study participants with suspected UTI were assessed for APN using ultrasound. Parenchymal echogenicity changes, renal pelvis dilatation, and the presence of a focal suspected lesion were assessed using conventional grayscale ultrasound. The presence and location of a decreased perfusion area were evaluated using color Doppler ultrasound (CDUS) and CEUS. Agreement between each ultrasound examination and a 99mTc‒dimercaptosuccinic acid (DMSA) scan was assessed using the κ value, and the most visible period of the lesion was evaluated using CEUS.ResultsThis study enrolled 21 participants (median age, 8.0 months; range, 2.0-61.0 months) with isolated urinary tract pathogens. Five increased parenchymal echotextures (11.9%) and 14 renal pelvic dilatations (33.3%) were confirmed, but no focal lesions were detected on the grayscale images. CDUS and CEUS showed decreased local perfusion suggestive of APN in two and five kidneys, respectively. DMSA scan showed substantial agreement with CEUS findings (κ = 0.80, P = 0.010), but other grayscale and CDUS findings did not agree with DMSA scan results (P > 0.05). All lesions were best observed in the late parenchymal phase on CEUS.ConclusionCEUS can reveal renal perfusion defects in pediatric patients with suspected APN without radiation exposure or sedation; therefore, CEUS may be a feasible and valuable diagnostic technique.

BackgroundCerebellar hemorrhage (CBH) is a major form of cerebellar injury in preterm infants. We aimed to investigate the risk factors and neurodevelopmental outcomes of isolated CBH and performed volumetric analysis at term-equivalent age.MethodsThis single-centered nested case-control study included 26 preterm infants with isolated CBH and 52 infants without isolated CBH and any significant supratentorial injury.ResultsIsolated CBH was associated with PCO2 fluctuation within 72 h after birth (adjusted odds ratio 1.007, 95% confidence interval 1.000–1.014). The composite score in the motor domain of the Bayley Scales of Infant and Toddler Development at 24 month of corrected age was lower in the punctate isolated CBH group than that in the control group (85.3 vs. 94.5, P = 0.023). Preterm infants with isolated CBH had smaller cerebellum and pons at term-equivalent age compared to the control group. Isolated CBH with adverse neurodevelopment had a smaller ventral diencephalon and midbrain compared to isolated CBH without adverse neurodevelopmental outcomes.ConclusionsIn preterm infants, isolated CBH with punctate lesions were associated with abnormal motor development at 24 months of corrected age. Isolated CBH accompanied by a smaller ventral diencephalon and midbrain at term equivalent had adverse neurodevelopmental outcomes.

Purpose This study evaluated the diagnostic performance of contrast-enhanced voiding urosonography (ce-VUS) using a second-generation ultrasound contrast agent for the diagnosis of vesicoureteral reflux (VUR) and intrarenal reflux (IRR), and compared it with that of standard fluoroscopic voiding cystourethrography (VCUG). Methods Thirty-two consecutive children from April to October 2019 were included in this study. ce-VUS and VCUG were performed simultaneously by two operators with intravesical infusion of a mixture of ultrasound contrast medium, iodinated contrast medium and water. Two pediatric radiologists independently reviewed the ce-VUS and VCUG images and reported the presence and degree of VUR (grades I-V), and the presence and type of IRR. Results Twenty-seven of 63 urinary systems showed VUR. Interobserver agreement for VUR grading was very good for both examinations (κ=0.87; 95% confidence interval [CI], 0.82 to 0.92 for ce-VUS and κ=0.92; 95% CI, 0.87 to 0.96 for VCUG). The detection rate of VUR showed no significant difference between the two examinations (P=0.370). Four cases of VUR were missed on ce-VUS, while one case of VUR was missed on VCUG. All four false-negative cases on ce-VUS were grade 1 VUR. The two examinations showed very good agreement regarding VUR grading (κ =0.89; 95% CI, 0.81 to 0.96). IRR was more frequently detected with ce-VUS than with VCUG (10 cases with ce-VUS vs. 3 cases with VCUG, P=0.016). Conclusion ce-VUS showed very good agreement with VCUG for detecting grade 2 VUR and above, while grade 1 VUR was sometimes missed with ce-VUS. IRR was more frequently detected with ce-VUS than with VCUG.

Abstract This study aimed to evaluate a deep learning model for generating synthetic contrast-enhanced CT (sCECT) from non-contrast chest CT (NCCT). A deep learning model was applied to generate sCECT from NCCT. We collected three separate data sets, the development set (n = 25) for model training and tuning, test set 1 (n = 25) for technical evaluation, and test set 2 (n = 12) for clinical utility evaluation. In test set 1, image similarity metrics were calculated. In test set 2, the lesion contrast-to-noise ratio of the mediastinal lymph nodes was measured, and an observer study was conducted to compare lesion conspicuity. Comparisons were performed using the paired t-test or Wilcoxon signed-rank test. In test set 1, sCECT showed a lower mean absolute error (41.72 vs 48.74; P

Magnetic Resonance Imaging (MRI) is sensitive to motion caused by patient movement due to the relatively long data acquisition time. This could cause severe degradation of image quality and therefore affect the overall diagnosis. In this paper, we develop an efficient retrospective 2D deep learning method called stacked U-Nets with self-assisted priors to address the problem of rigid motion artifacts in 3D brain MRI. The proposed work exploits the usage of additional knowledge priors from the corrupted images themselves without the need for additional contrast data. The proposed network learns the missed structural details through sharing auxiliary information from the contiguous slices of the same distorted subject. We further design a refinement stacked U-Nets that facilitates preserving the spatial image details and improves the pixel-to-pixel dependency. To perform network training, simulation of MRI motion artifacts is inevitable. The proposed network is optimized by minimizing the loss of structural similarity (SSIM) using the synthesized motion-corrupted images from 83 real motion-free subjects. We present an intensive analysis using various types of image priors: the proposed self-assisted priors and priors from other image contrast of the same subject. The experimental analysis proves the effectiveness and feasibility of our self-assisted priors since it does not require any further data scans. The overall image quality of the motion-corrected images via the proposed motion correction network significantly improves SSIM from 71.66% to 95.03% and declines the mean square error from 99.25 to 29.76. These results indicate the high similarity of the brain's anatomical structure in the corrected images compared to the motion-free data. The motion-corrected results of both the simulated and real motion data showed the potential of the proposed motion correction network to be feasible and applicable in clinical practices.

Abstract Background Pediatric patients with liver disease require noninvasive monitoring to evaluate the risk of fibrosis progression. This study aimed to identify the significant factors affecting liver stiffness values using two-dimensional shear-wave elastography (2D-SWE), and determine whether liver stiffness can predict the fibrosis stage of various childhood liver diseases. Methods This study included 30 children (22 boys and 8 girls; mean age, 5.1 ± 6.1 years; range, 7 days–17.9 years) who had undergone biochemical evaluation, 2D-SWE examination, histopathologic analysis of fibrosis grade (F0 to F3), assessment of necroinflammatory activity, and steatosis grading between August 2016 and March 2020. The liver stiffness from 2D-SWE was compared between fibrosis stages using Kruskal–Wallis analysis. Factors that significantly affected liver stiffness were evaluated using univariate and multivariate linear regression analyses. The diagnostic performance was determined from the area under the receiver operating curve (AUC) values of 2D-SWE liver stiffness. Results Liver stiffness at the F0-1, F2, and F3 stages were 7.9, 13.2, and 21.7 kPa, respectively (P

Background: The inter- and intrarater variability of conventional computed tomography (CT) classification systems for evaluating the extent of ischemic-edematous insult following traumatic brain injury (TBI) may hinder the robustness of TBI prognostic models.Objective: This study aimed to employ fully automated quantitative densitometric CT parameters and a cutting-edge machine learning algorithm to construct a robust prognostic model for pediatric TBI.Methods: Fifty-eight pediatric patients with TBI who underwent brain CT were retrospectively analyzed. Intracranial densitometric information was derived from the supratentorial region as a distribution representing the proportion of Hounsfield units. Furthermore, a machine learning-based prognostic model based on gradient boosting (i.e., CatBoost) was constructed with leave-one-out cross-validation. At discharge, the outcome was assessed dichotomously with the Glasgow Outcome Scale (favorability: 1–3 vs. 4–5). In-hospital mortality, length of stay (>1 week), and need for surgery were further evaluated as alternative TBI outcome measures.Results: Densitometric parameters indicating reduced brain density due to subtle global ischemic changes were significantly different among the TBI outcome groups, except for need for surgery. The skewed intracranial densitometry of the unfavorable outcome became more distinguishable in the follow-up CT within 48 h. The prognostic model augmented by intracranial densitometric information achieved adequate AUCs for various outcome measures [favorability = 0.83 (95% CI: 0.72–0.94), in-hospital mortality = 0.91 (95% CI: 0.82–1.00), length of stay = 0.83 (95% CI: 0.72–0.94), and need for surgery = 0.71 (95% CI: 0.56–0.86)], and this model showed enhanced performance compared to the conventional CRASH-CT model.Conclusion: Densitometric parameters indicative of global ischemic changes during the acute phase of TBI are predictive of a worse outcome in pediatric patients. The robustness and predictive capacity of conventional TBI prognostic models might be significantly enhanced by incorporating densitometric parameters and machine learning techniques.

PurposeThe purpose of this study was to identify imaging risk factors on contrast-enhanced hip MRI after closed reduction of developmental dysplasia of the hip (DDH) that could predict future development of avascular necrosis (AVN) of the femoral head.Materials and methodsFifty-eight infants (F: M = 53: 5, aged 3-18 months) who underwent immediate postoperative contrast-enhanced hip MRI after closed reduction of DDH were included in this study. Quality of reduction (concentric vs eccentric reduction with or without obstacles), abduction angle of the hip, presence of ossific nucleus, and pattern of contrast enhancement of the femoral head were retrospectively evaluated on MRI. Interobserver agreement of contrast enhancement pattern on MRI were evaluated by two radiologists. Development of AVN was determined through radiographic findings at 1 year after reduction.ResultsAVN of the femoral head developed in 13 (22%) of 58 patients. Excessive abduction of the hip joint (OR 4.65, [95% CI 1.20, 18.06] and global decreased enhancement of the femoral head (OR 71.66, [95% CI 10.54, 487.31]) exhibited statistically significant differences between the AVN and non-AVN groups (P < 0.05). Eccentric reduction (P = 0.320) did not show statistically significant difference between two groups and invisible ossific nucleus (P = 0.05) showed borderline significance. Multi-variable logistic regression indicated that global decreased enhancement of the femoral head was a significant risk factor of AVN (OR 27.92, 95% CI [4.17, 350.18]) (P = 0.0031). Interobserver agreement of contrast enhancement pattern analysis and diagnosis of AVN were good (0.66, 95% CI [0.52, 0.80]).ConclusionContrast-enhanced hip MRI provides accurate anatomical assessment of the hip after closed reduction of DDH. Global decreased enhancement of the femoral head could be used as a good predictor for future development of AVN after closed reduction of DDH.

Abstract Background Multicentric carpotarsal osteolysis syndrome (MCTO) is characterized by progressive destruction and disappearance of the carpal and tarsal bones associated with nephropathy. MCTO is caused by loss-of-function mutations in the MAF bZIP transcription factor B (MAFB) gene. Case presentation This report describes three unrelated patients with MAFB mutations, including two male and one female patient. Osteolytic lesions in the carpal and tarsal bones were detected at 2 years, 12 years, and 14 months of age, respectively. Associated proteinuria was noted at 4 years, 12 years, and 3 months of age, respectively. Kidney biopsy was performed in two of them and revealed focal segmental glomerulosclerosis (FSGS). One patient showed progression to end-stage renal disease, that is by 1 year after the detection of proteinuria. The second patient had persistent proteinuria but maintained normal renal function. In the third patient, who did not undergo a kidney biopsy, the proteinuria disappeared spontaneously. The bony lesions worsened progressively in all three patients. Mutational study of MAFB revealed three different mutations, two novel mutations [c.183C > A (p.Ser61Arg) and c.211C > G (p.Pro71Ala)] and one known mutation [c.212C > T (p.Pro71Leu)]. Conclusion We report three cases with MCTO and two novel MAFB mutations. The renal phenotypes were different among the three patients, whereas progressive worsening of the bony lesions was common in all patients. We also confirmed FSGS to be an early renal pathologic finding in two cases. A diagnosis of MCTO should be considered in patients with progressive bone loss concentrated primarily in the carpal and tarsal bones and kidney involvement, such as proteinuria.

Purpose The purpose of this study was to evaluate the usefulness of the stability index (SI) in liver stiffness measurements using shear wave elastography (SWE) in children. Methods A total of 29 children and young adults (mean age, 16.1 years; range, 8 to 28 years; 11 boys and 18 girls) who underwent liver stiffness measurements using SWE under free-breathing and breath-holding conditions were included in our study. Ten SWE measurements were acquired in each of four groups: free-breathing and breath-holding, and with and without using the SI. The failure rate of acquisition of SI values over 90% was calculated in each group. To evaluate variability in the SWE measurements, the standard deviation, coefficient of variation, and percentage of unreliable measurements were compared. Intraobserver agreement and the optimal minimal number of measurements were calculated using intraclass correlation coefficients. Results A failure to acquire SI values over 90% was observed in 17% of the scans in the free-breathing group and in 7% of the scans in the breath-holding group. In both groups, utilizing the SI led to a significantly lower standard deviation and coefficient of variation. When using the SI, the percentage of unreliable measurements decreased from 16.7% to 8.3% in the free-breathing group and 14.8% to 0% in the breath-holding group. With the use of the SI, intraobserver agreement increased and the optimal minimal number of repeated measurements decreased in both the free-breathing and breath-holding groups. Conclusion Utilization of the SI in the measurement of liver SWE in children reduced measurement variability and increased reliability in both free-breathing and breath-holding conditions.

Background: Systemic infection is a major upstream mechanism for white matter abnormality (WMA). Our aim was to evaluate the risk factors for moderate-to-severe WMA in extremely premature infants (gestational age

Purpose The aim of this study was to evaluate impact of 3-dimensional cranial ultrasonography (3DUS) on reinterpretation of cranial ultrasonography images in neonates in comparison with 2-dimensional cranial ultrasonography (2DUS). Methods We retrospectively enrolled 50 young infants who simultaneously underwent both 2DUS and 3DUS scanning from February to March 2015. Two pediatric radiologists independently reviewed both scans for overall image quality on a 5-point scale. Five features were evaluated in both scans: the presence of germinal matrix hemorrhage (GMH), intraventricular hemorrhage (IVH), ventriculomegaly (VM), abnormality of periventricular echogenicity (PVE), and focal parenchymal lesions (FL). The concordance rate between the two scanning modes was calculated. The confidence level for each finding on a 3-point scale and the scanning time were compared between the two scanning modes. Interobserver agreement was evaluated using kappa statistics. Results Both scans demonstrated similar overall image quality in terms of reinterpretation (mean scores for 2DUS and 3DUS, 4.0±0.5 and 4.0±0.7 in reviewer 1, 3.9±0.6 and 4.0±0.8 in reviewer 2, respectively). GMH, IVH, VM, and FL showed perfect concordance, while PVE showed a concordance rate of 91.4% between the two modes by both reviewers. 3DUS was associated with a higher diagnostic confidence in the evaluation of GMH, IVH, and FL than 2DUS (P

Purpose The purpose of this study was to identify ultrasonographic features that can be used to differentiate between thyroglossal duct cysts (TGDCs) and dermoid cysts (DCs). Methods We searched surgical pathology reports completed between January 2004 and October 2015 and identified 66 patients with TGDCs or DCs who had undergone preoperative ultrasonography. The ultrasound images were reviewed by two radiologists who were blinded to the pathological diagnosis. They evaluated the following parameters: dimensions, shape, margin, location in relation to the midline, level in relation to the hyoid bone, attachment to the hyoid bone, the depth of the lesion in relation to the strap muscles, internal echogenicity, internal echogenic dots, multilocularity, the presence of a longitudinal extension into the tongue base, posterior acoustic enhancement, the presence of internal septae, and intralesional vascularity. Results There were 50 TGDCs and 16 DCs. TGDCs were significantly more likely than DCs to have an irregular shape, an ill-defined margin, attachment to the hyoid bone, an intramuscular location, heterogeneous internal echogenicity, multilocularity, and longitudinal extension into the tongue base. Conclusion Ultrasound findings may inform the differential diagnosis between TGDCs and DCs.

Pulmonary alveolar proteinosis (PAP) is a rare disease in children characterized by intra-alveolar accumulation of surfactant proteins, which severely reduces gaseous exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. Herein, we present a pediatric case of PAP treated with WLL. An 11-year-old boy was admitted with the chief complaint of a dry cough lasting 6 months. He developed symptoms of dyspnea on exertion and had difficulty in climbing stairs. He was ultimately diagnosed with PAP through video-assisted thoracoscopic lung biopsy. As first-line of treatment for PAP, he underwent therapeutic WLL for each of his lungs on separate days. After a brief recovery, his symptoms gradually worsened; therefore, he underwent a second WLL. This is the first pediatric case of PAP relapse despite successful WLL in Korea.

Acute hypersensitivity reactions to gadolinium-based magnetic resonance (MR) contrast media have been shown to have a much lower incidence and they are generally milder in terms of severity than acute adverse reactions associated with the use of iodinated contrast media for computed tomography scans. However, even though it is rare, a severe hypersensitivity reaction to MR contrast media can occur. Here we present the case of a 66-year-old woman who experienced a severe hypersensitivity reaction after administration of gadolinium-based contrast media without a previous history of allergies.

OBJECTIVE:To retrospectively evaluate the value of computerized 3D texture analysis for differentiating pulmonary metastases from non-metastatic lesions in pediatric patients with osteosarcoma. MATERIALS AND METHODS:This retrospective study was approved by the institutional review board. The study comprised 42 pathologically confirmed pulmonary nodules in 16 children with osteosarcoma who had undergone preoperative computed tomography between January 2009 and December 2014. Texture analysis was performed using an in-house program. Multivariate logistic regression analysis was performed to identify factors for differentiating metastatic nodules from non-metastases. A subgroup analysis was performed to identify differentiating parameters in small non-calcified pulmonary nodules. The receiver operator characteristic curve was created to evaluate the discriminating performance of the established model. RESULTS:There were 24 metastatic and 18 non-metastatic lesions. Multivariate analysis revealed that higher mean attenuation (adjusted odds ratio [OR], 1.014, P = 0.003) and larger effective diameter (OR, 1.745, P = 0.012) were significant differentiators. The analysis with small non-calcified pulmonary nodules (7 metastases and 18 non-metastases) revealed significant inter-group differences in various parameters. Logistic regression analysis revealed that higher mean attenuation (OR, 1.007, P = 0.008) was a significant predictor of non-calcified pulmonary metastases. The established logistic regression model of subgroups showed excellent discriminating performance in the ROC analysis (area under the curve, 0.865). CONCLUSION:Pulmonary metastases from osteosarcoma could be differentiated from non-metastases by using computerized texture analysis. Higher mean attenuation and larger diameter were significant predictors for pulmonary metastases, while higher mean attenuation was a significant predictor for small non-calcified pulmonary metastases.

BackgroundRadiologic evaluation of children with Mycoplasma pneumoniae is important for diagnosis and management.ObjectiveTo investigate the correlation between chest radiographic findings and the clinical features in children with Mycoplasma pneumoniae pneumonia.Materials and methodsThis study included 393 hospitalized children diagnosed with M. pneumoniae pneumonia between January 2000 and August 2016. Their clinical features and chest radiographs were reviewed. Radiographic findings were categorized and grouped as consolidation group (lobar or segmental consolidation) and non-consolidation group (patchy infiltration, localized reticulonodular infiltration, or parahilar peribronchial infiltration).ResultsLobar or segmental consolidation (37%) was the most common finding, followed by parahilar or peribronchial infiltration (27%), localized reticulonodular infiltration (21%) and patchy infiltration (15%). The consolidation group was more frequently accompanied by pleural effusions (63%), compared to the non-consolidation group (16%). Compared with patients in the non-consolidation group, those in the consolidation group were associated with a significantly higher rate of hypoxia, tachypnea, tachycardia, extrapulmonary manifestations, prolonged fever, and longer periods of anti-mycoplasma therapy and hospitalization. Lobar or segmental consolidation was significantly more frequent in children ≥5 years old (44%) compared with children 2-5 years old (34%) and ConclusionThe chest radiographic findings of children with M. pneumoniae pneumonia correlate well with the clinical features. Consolidative lesions were frequently observed in older children and were associated with more severe clinical features.

Widespread use of fetal ultrasonography is accompanied by more frequent detection of antenatal hydronephrosis. Therefore, sonographic evaluation of neonates with a history of antenatal hydronephrosis is becoming more widespread. As an initial postnatal non-invasive imaging modality, ultrasonography is used to screen for persistence of hydronephrosis, determine the level and severity of obstruction, and contribute to appropriate diagnosis and treatment. This review aims to provide a practical overview of the sonographic evaluation of neonatal hydronephrosis and to describe the sonographic findings of conditions associated with hydronephrosis in the newborn.

To evaluate the patients' morphologic factors affecting radiation dose in pediatric chest CT.From November 2013 to May 2015, 315 pediatric chest CT scans were obtained using a CT scanner, and classified into 5 groups according to the patients' age. For each age group, the chest CT scans were divided into two subgroups. A cut-off value used was the 75th percentile of size-specific dose estimates (SSDE), age-specific diagnostic reference level (DRL): less than the 75th percentile of SSDE (Group A, n = 238) and greater than the 75th percentile of SSDE (Group B, n = 77). All CT scans were performed with the same protocol using automatic tube voltage selection and current modulation techniques. The morphologic factors of the patients including body mass index (BMI), arm angles, presence of medical devices in the scan field, and degree of off-centering within the CT gantry were compared between groups A and B.Group B showed narrower arm angles on scout and coronal reformatted images, higher frequency of the presence of devices and higher BMI than group A (P < 0.001, P < 0.001; P = 0.018, and P < 0.001, respectively). In multivariate analysis, narrower arm angles, the presence of devices on the scout images and higher BMI were independently associated with higher SSDE (P = 0.001, P = 0.037, and P < 0.001, respectively).During acquisition of the scout images, arms-down position and the presence of medical devices were associated with a high radiation dose above age-specific DRLs in pediatric chest CT, regardless of repositioning before the actual scanning. In addition, off-centering had no clinical impact on radiation dose in the routine practice.

To evaluate the incremental value of a combination of magnetic resonance cholangiopancreatography (MRCP) and ultrasonography (US), compared to US alone, for diagnosing biliary atresia (BA) in neonates and young infants with cholestasis.The institutional review board approved this retrospective study. The US and MRCP studies were both performed on 64 neonates and young infants with BA (n = 41) or without BA (non-BA) (n = 23). Two observers reviewed independently the US alone set and the combined US and MRCP set, and graded them using a five-point scale. Diagnostic performance was compared using pairwise comparison of the receiver operating characteristics (ROC) curve. The sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value were assessed.The diagnostic performance (the area under the ROC curve [Az]) for diagnosing BA improved significantly after additional review of MRCP images; Az improved from 0.688 to 0.901 (P = .015) for observer 1 and from 0.676 to 0.901 (P = .011) for observer 2. The accuracy of MRCP combined with US (observer 1, 95% [61/64]; observer 2 92% [59/64]) and PPV (observer 1, 95% [40/42]; observer 2 91% [40/44]) were significantly higher than those of US alone for both observers (accuracy: observer 1, 73% [47/64], P = 0.003; observer 2, 72% [46/64], P = 0.004; PPV: observer 1, 76% [35/46], P = 0.016; observer 2, 76% [34/45], P = 0.013). Interobserver agreement of confidence levels was good for US alone (ĸ = 0.658, P < .001) and was excellent for the combined set of US and MRCP (ĸ = 0.929, P < .001).Better diagnostic performance was achieved with the combination of US and MRCP than with US alone for the evaluation of BA in neonates and young infants with cholestasis.

Auraptene is the most abundant naturally occurring geranyloxycoumarin. It is primarily isolated from plants belonging to the Rutaceae family, many of which, such as citrus fruits, are used as food in many countries. Auraptene is a biologically active secondary metabolite that possesses valuable properties. The aim of this study was to investigate the in vitro inhibitory effects of auraptene on melanogenesis and the enzymes associated with it, such as tyrosinase, tyrosinase-related protein (TRP)-1, and TRP-2, in B16F10 murine melanoma cells. We found that auraptene significantly attenuated melanin synthesis and reduced the activity of intracellular tyrosinase, which was the rate-limiting melanogenic enzyme. Western blotting analysis showed that auraptene decreased tyrosinase and TRP-2 protein expression. In addition, auraptene significantly decreased the expression of microphthalmia-associated transcription factor (MITF), a key regulator of melanogenesis. Extracellular signal-regulated kinase (ERK) activation has been reported to be involved in the inhibition of melanogenesis. Thus, we next investigated if the hypopigmentary effects of auraptene were related to the activation of ERK. Auraptene was found to induce phosphorylation of ERK in a dose-dependent manner. Our results suggest that auraptene inhibits melanogenesis by activating the ERK pathway-mediated suppression of MITF and its downstream target genes, including tyrosinase. Therefore, auraptene may be used as a whitening agent in the development of functional cosmetics.

This study presents a new density filter for a pipe-shaped structure and its application to fluid topology optimization. A simple and straight pipe-shaped structure for fluid is preferred for many engineering purposes rather than the perplex manifold structure provided by the topology optimization method. To determine an optimal pipe structure for fluid, we develop a new density filter and apply it to fluid topology optimization. Hence, the original spatially varying design variables of the fluid topology optimization are modified based on the pipe density filter. Subsequently, the filter design variables, including a uniform pipe wall thickness and adjusted cross-section, are used for artificial pseudo-rigid bodies in fluid topology optimization. An additional constraint is imposed to maintain a nearly uniform pipe thickness. Several numerical examples are solved to demonstrate the validity of the present pipe density filter for fluid topology optimization problems minimizing the energy dissipation of the fluid and controlling the particles suspended in the fluid. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to predict high-risk neuroblastoma among neuroblastic tumors using radiomics features extracted from MRI. Materials and Methods: Pediatric patients (age≤18 years) diagnosed with neuroblastic tumors who had pre-treatment MR images available were enrolled from institution A from January 2010 to November 2019 (training set) and institution B from January 2016 to January 2022 (test set). Segmentation was performed with regions of interest manually drawn along tumor margins on the slice with the widest tumor area by two radiologists. First-order and texture features were extracted and intraclass correlation coefficients (ICCs) were calculated. Multivariate logistic regression (MLR) and random forest (RF) models from 10-fold cross-validation were built using these features. The trained MLR and RF models were tested in an external test set. Results: Thirty-two patients (M:F=23:9, 26.0±26.7 months) were in the training set and 14 patients (M:F=10:4, 33.4±20.4 months) were in the test set with radiomics features (n=930) being extracted. For 10 of the most relevant features selected, intra- and interobserver variability was moderate to excellent (ICCs 0.633–0.911, 0.695–0.985, respectively). The area under the receiver operating characteristic curve (AUC) was 0.94 (sensitivity 67%, specificity 91%, and accuracy 84%) for the MLR model and the average AUC was 0.83 (sensitivity 44%, specificity 87%, and accuracy 75%) for the RF model from 10-fold cross-validation. In the test set, AUCs of the MLR and RF models were 0.94 and 0.91, respectively. Conclusion: An MRI-based radiomics model can help predict high-risk neuroblastoma among neuroblastic tumors. [ABSTRACT FROM AUTHOR]