Monitoring Posterior Cerebral Perfusion Changes With Dynamic Susceptibility Contrast-Enhanced Perfusion MRI After Anterior Revascularization Surgery in Pediatric Moyamoya Disease

Objective@#To determine whether dynamic susceptibility contrast-enhanced (DSC) perfusion magnetic resonance imaging (MRI) can be used to evaluate posterior cerebral circulation in pediatric patients with moyamoya disease (MMD) who underwent anterior revascularization. @*Materials and Methods@#This study retrospectively included 73 patients with MMD who underwent DSC perfusion MRI (age, 12.2 ± 6.1 years) between January 2016 and December 2020, owing to recent-onset clinical symptoms during the follow-up period after completion of anterior revascularization. DSC perfusion images were analyzed using a dedicated software package (NordicICE; Nordic NeuroLab) for the middle cerebral artery (MCA), posterior cerebral artery (PCA), and posterior border zone between the two regions (PCA-MCA). Patients were divided into two groups; the PCA stenosis group included 30 patients with newly confirmed PCA involvement, while the no PCA stenosis group included 43 patients without PCA involvement. The relationship between DSC perfusion parameters and PCA stenosis, as well as the performance of the parameters in discriminating between groups, were analyzed. @*Results@#In the PCA stenosis group, the mean follow-up duration was 5.3 years after anterior revascularization, and visual disturbances were a common symptom. Normalized cerebral blood volume was increased, and both the normalized time-topeak (nTTP) and mean transit time values were significantly delayed in the PCA stenosis group compared with those in the no PCA stenosis group in the PCA and PCA-MCA border zones. TTP PCA (odds ratio [OR] = 6.745; 95% confidence interval [CI] = 2.665–17.074; P < 0.001) and CBVPCA-MCA (OR = 1.567; 95% CI = 1.021–2.406; P = 0.040) were independently associated with PCA stenosis. TTPPCA showed the highest receiver operating characteristic curve area in discriminating for PCA stenosis (0.895; 95% CI = 0.803–0.986). @*Conclusion@#nTTP can be used to effectively diagnose PCA stenosis. Therefore, DSC perfusion MRI may be a valuable tool for monitoring PCA stenosis in patients with MMD.

Development and Evaluation of Deep Learning-Based Automatic Segmentation Model for Skull Zero TE MRI in Children

Purpose@#To develop and evaluate a deep learning technique to automatically segment bone structures in zero echo time (ZTE) for skull magnetic resonance imaging (MRI) in children. @*Materials and Methods@#From January to December 2021, 38 bone ZTE MRIs from infants and children (age range, 1–31 months) were collected for model development.Mask images were generated by manually segmenting the craniofacial bone using a commercial segmentation program. Among them, 35 ZTE series were used to train the three-dimensional (3D)-nnUnet deep learning model and the remaining three series were used for model validation. A temporally different dataset of 19 ZTE bone MRIs obtained in May 2022 from infants and children (age range, 3–168 months) was used to determine the model’s performance. Dice similarity coefficient was calculated for each test case.From 3D volume rendering images, segmentation accuracy, overall image quality, and visibility of cranial sutures were subjectively evaluated on a 5-point scale and compared with ground truth data from manual segmentation. Reasons for segmentation failure were analyzed using axially segmented ZTE images. @*Results@#For the test set, the mean Dice similarity coefficient was 0.985 ± 0.019. The segmentation accuracy was lower than the ground truth without showing a statistically significant difference between the two (3.39 ± 1.11 vs. 3.73 ± 0.77, p = 0.055). The overall image quality and suture visibility showed no significant difference (3.34 ± 0.75 vs.3.42 ± 0.69, p = 0.317; 3.55 ± 0.97 vs. 3.60 ± 0.95, p = 0.157). Common reasons for low segmentation accuracy were well-pneumatized sinuses, metal artifacts, skin at the vertex level, and bones too thin. @*Conclusion@#The deep learning-based automatic segmentation technique of bone ZTE MRIs showed comparable segmentation performance to manual segmentation. Using the deep learning-based segmentation results, acceptable 3D-volume rendering images of craniofacial bones were generated.

Cerebrovascular Arteriopathy in Microcephalic Osteodysplastic Primordial Dwarfism Type II

Microcephalic osteodysplastic primordial dwarfism type II (MOPD-II) is a rare disease with characteristic skeletal abnormalities and severe comorbidities related to cerebrovascular diseases. It is frequently associated with early onset cerebrovascular diseases due to its predisposition to intracranial arteriopathies such as aneurysms and moyamoya syndrome. Herein, we report cases of two siblings presenting with multiple cerebral aneurysms and moyamoya syndrome. Two brothers with short stature were genetically diagnosed with MOPD-II at the age of 18 years and 9 years. Magnetic resonance angiography of the brother at the age of 20 years demonstrated a ruptured left A2 aneurysm with multiple variable-sized aneurysms in intracerebral arteries. The younger brother underwent brain imaging for screening at the age of 12 years which revealed a sizable basilar top aneurysm, multiple tiny aneurysms, and steno-occlusive changes in the left A1 and M1. In conclusion, cerebrovascular arteriopathy was diagnosed using brain magnetic resonance imaging in a pediatric patient with MOPD-II. Cerebrovascular comorbidities can occur at any point in life. It can lead to disability or death. Therefore, routine screening for cerebrovascular comorbidities in patients with MOPD-II who have reached adolescence is recommended.

Usefulness of two-dimensional shear wave elastography in diagnosing hepatic veno-occlusive disease in pediatric patients undergoing hematopoietic stem cell transplantation

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<0.001). Liver stiffness values above 7.2 kPa showed 84.2% sensitivity and 93.3% specificity in distinguishing VOD from non-VOD (area under the curve, 0.925; 95% confidence interval, 0.780 to 0.987; P<0.001). A subgroup analysis of 11 patients showed a linear decrease in liver stiffness values after VOD diagnosis with treatment (first, second, and third follow-ups; 13.5±1.7, 11.3±1.4, and 9.5±0.8 kPa, respectively), but without statistical significance in the pairwise analysis. @*Conclusion@#Liver stiffness measured using 2D-SWE increased in pediatric patients who develop VOD after HSCT. Therefore, liver stiffness can be a predictive and quantitative parameter for diagnosing VOD.

Deep Learning-Based Computed Tomography Image Standardization to Improve Generalizability of Deep Learning-Based Hepatic Segmentation

Objective@#We aimed to investigate whether image standardization using deep learning-based computed tomography (CT) image conversion would improve the performance of deep learning-based automated hepatic segmentation across various reconstruction methods. @*Materials and Methods@#We collected contrast-enhanced dual-energy CT of the abdomen that was obtained using various reconstruction methods, including filtered back projection, iterative reconstruction, optimum contrast, and monoenergetic images with 40, 60, and 80 keV. A deep learning based image conversion algorithm was developed to standardize the CT images using 142 CT examinations (128 for training and 14 for tuning). A separate set of 43 CT examinations from 42 patients (mean age, 10.1 years) was used as the test data. A commercial software program (MEDIP PRO v2.0.0.0, MEDICALIP Co. Ltd.) based on 2D U-NET was used to create liver segmentation masks with liver volume. The original 80 keV images were used as the ground truth. We used the paired t-test to compare the segmentation performance in the Dice similarity coefficient (DSC) and difference ratio of the liver volume relative to the ground truth volume before and after image standardization. The concordance correlation coefficient (CCC) was used to assess the agreement between the segmented liver volume and ground-truth volume. @*Results@#The original CT images showed variable and poor segmentation performances. The standardized images achieved significantly higher DSCs for liver segmentation than the original images (DSC [original, 5.40%–91.27%] vs. [standardized, 93.16%–96.74%], all P < 0.001). The difference ratio of liver volume also decreased significantly after image conversion (original, 9.84%–91.37% vs. standardized, 1.99%–4.41%). In all protocols, CCCs improved after image conversion (original, -0.006–0.964 vs. standardized, 0.990–0.998). @*Conclusion@#Deep learning-based CT image standardization can improve the performance of automated hepatic segmentation using CT images reconstructed using various methods. Deep learning-based CT image conversion may have the potential to improve the generalizability of the segmentation network.

Do-It-Yourself Open Artificial Pancreas System in Children and Adolescents with Type 1 Diabetes Mellitus: Real-World Data

Few studies have been conducted among Asian children and adolescents with type 1 diabetes mellitus (T1DM) using do-it-yourself artificial pancreas system (DIY-APS). We evaluated real-world data of pediatric T1DM patients using DIY-APS. Data were obtained for 10 patients using a DIY-APS with algorithms. We collected sensor glucose and insulin delivery data from each participant for a period of 4 weeks. Average glycosylated hemoglobin was 6.2%±0.3%. The mean percentage of time that glucose level remained in the target range of 70 to 180 mg/dL was 82.4%±7.8%. Other parameters including time above range, time below range and mean glucose were also within the recommended level, similar to previous commercial and DIY-APS studies. However, despite meeting the target range, unadjusted gaps were still observed between the median basal setting and temporary basal insulin, which should be handled by healthcare providers.

Clinical Results of Punch Biopsy for Various Eyelid Disorders in the Office Setting

Purpose@#To introduce punch biopsy, which can be easily performed in an outpatient setting, and to evaluate its clinical usefulness. @*Methods@#A retrospective study was performed in 33 patients with atypical eyelid disorders who underwent punch biopsy. @*Results@#This study included 33 eyes (33 patients; male:female = 12:21) with a mean age of 57.27 ± 19.12 years. There were 19 (57.6%) right eye, 14 (42.4%) left eye, 15 (45.5%) upper eyelid, and 18 (54.5%) lower eyelid lesions. The biopsy approaches were percutaneous and transconjunctival in 24 (72.7%) and nine (27.3%) eyes, respectively. Benign and malignant lesions were found in 21 (63.6%) and 12 (36.4%) eyes, respectively. The benign lesions included chalazion (52.4%), acute and chronic inflammation (23.8%), acanthosis (4.8%), and ectopic cillia (4.8%), and were treated conservatively by intralesional steroid injections or incision and drainage. The malignant lesions included sebaceous carcinomas (33.3%), squamous cell carcinomas (25%), and basal cell carcinomas (16.7%), with various treatments, including surgical resections, chemotherapy, and radiation therapy. @*Conclusions@#Punch biopsy is a safe and quick procedure that can easily be performed in the office setting for patients who require a pathologic diagnosis for eyelid diseases, including malignancies. It may be useful for diagnosis and treatment planning.

Operational Definition Identifying Osteoporotic Vertebral Fractures in the Claims Database

Background@#We analyzed the International Classification of Diseases, 10th edition (ICD-10) diagnostic codes, procedure codes, and radiographic image codes for vertebral fracture (VF) used in the database of Health Insurance Review and Assessment Service (HIRA) of Korea to establish a validated operational definition for identifying patients with osteoporotic VF in claims data. @*Methods@#We developed three operational definitions for detecting VFs using 9 diagnostic codes, 5 procedure codes and 4 imaging codes. Medical records and radiographs of 2,819 patients, who had primary and subordinated codes of VF between January 2016 and December 2016 at two institutions, were reviewed to detect true vertebral fractures. We evaluated the sensitivity and positive predictive value (PPV) of the operational definition in detecting true osteoporotic VF and obtained the receiver operating characteristic (ROC) curve. @*Results@#Among the 2,819 patients who had primary or secondary diagnosis codes for VF, 995 patients satisfied at least one of the criteria for the operational definition of osteoporotic VF. Of these patients, 594 were judged as having true fractures based on medical records and radiographic examinations. The sensitivity and PPV were 62.5 (95% confidence interval [CI], 59.4–65.6) and 59.7(95% CI, 56.6–62.8) respectively. In the receiver operating characteristic analysis, area under the curve (AUC) was 0.706 (95% CI, 0.688–0.724). @*Conclusion@#Our findings demonstrate the validity of our operational definitions to identify VFs more accurately using claims data. This algorithm to identify VF is likely to be useful in future studies for diagnosing osteoporotic VF.

Inflammatory responses of C57BL/6NKorl mice to dextran sulfate sodium-induced colitis: comparison between three C57BL/6N sub-strains / 한국실험동물학회지

Background@#Inflammatory bowel disease (IBD), including both Crohn’s disease and ulcerative colitis, are chronic human diseases that are challenging to cure and are often unable to be resolved. The inbred mouse strain C57BL/ 6 N has been used in investigations of IBD as an experimental animal model. The purpose of the current study was to compare the inflammatory responsiveness of C57BL/6NKorl mice, a sub-strain recently established by the National Institute of Food and Drug Safety Evaluation (NIFDS), with those of C57BL/6 N mice from two different sources using a dextran sulfate sodium (DSS)-induced colitis model. @*Results@#Male mice (8 weeks old) were administered DSS (0, 1, 2, or 3%) in drinking water for 7 days. DSS significantly decreased body weight and colon length and increased the colon weight-to-length ratio. Moreover, severe colitisrelated clinical signs including diarrhea and rectal bleeding were observed beginning on day 4 in mice administered DSS at a concentration of 3%. DSS led to edema, epithelial layer disruption, inflammatory cell infiltration, and cytokine induction (tumor necrosis factor-α, interleukin-6, and interleukin-1β) in the colon tissues. However, no significant differences in DSS-promoted abnormal symptoms or their severity were found between the three sub-strains. @*Conclusions@#These results indicate that C57BL/6NKorl mice responded to DSS-induced colitis similar to the generally used C57BL6/N mice, thus this newly developed mouse sub-strain provides a useful animal model of IBD.

Monitoring Cerebral Perfusion Changes Using Arterial Spin-Labeling Perfusion MRI after Indirect Revascularization in Children with Moyamoya Disease

Objective@#To assess the role of arterial spin-labeling (ASL) perfusion MRI in identifying cerebral perfusion changes after indirect revascularization in children with moyamoya disease. @*Materials and Methods@#We included pre- and postoperative perfusion MRI data of 30 children with moyamoya disease (13 boys and 17 girls; mean age ± standard deviation, 6.3 ± 3.0 years) who underwent indirect revascularization between June 2016 and August 2017. Relative cerebral blood flow (rCBF) and qualitative perfusion scores for arterial transit time (ATT) effects were evaluated in the middle cerebral artery (MCA) territory on ASL perfusion MRI. The rCBF and relative time-topeak (rTTP) values were also measured using dynamic susceptibility contrast (DSC) perfusion MRI. Each perfusion change on ASL and DSC perfusion MRI was analyzed using the paired t test. We analyzed the correlation between perfusion changes on ASL and DSC images using Spearman’s correlation coefficient. @*Results@#The ASL rCBF values improved at both the ganglionic and supraganglionic levels of the MCA territory after surgery (p = 0.040 and p = 0.003, respectively). The ATT perfusion scores also improved at both levels (p < 0.001 and p < 0.001, respectively). The rCBF and rTTP values on DSC MRI showed significant improvement at both levels of the MCA territory of the operated side (all p < 0.05). There was no significant correlation between the improvements in rCBF values on the two perfusion images (r = 0.195, p = 0.303); however, there was a correlation between the change in perfusion scores on ASL and rTTP on DSC MRI (r = 0.701, p < 0.001). @*Conclusion@#Recognizing the effects of ATT on ASL perfusion MRI may help monitor cerebral perfusion changes and complement quantitative rCBF assessment using ASL perfusion MRI in patients with moyamoya disease after indirect revascularization.

Diagnostic Performance of a New Convolutional Neural Network Algorithm for Detecting Developmental Dysplasia of the Hip on Anteroposterior Radiographs

Objective@#To evaluate the diagnostic performance of a deep learning algorithm for the automated detection of developmental dysplasia of the hip (DDH) on anteroposterior (AP) radiographs. @*Materials and Methods@#Of 2601 hip AP radiographs, 5076 cropped unilateral hip joint images were used to construct a dataset that was further divided into training (80%), validation (10%), or test sets (10%). Three radiologists were asked to label the hip images as normal or DDH. To investigate the diagnostic performance of the deep learning algorithm, we calculated the receiver operating characteristics (ROC), precision-recall curve (PRC) plots, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) and compared them with the performance of radiologists with different levels of experience. @*Results@#The area under the ROC plot generated by the deep learning algorithm and radiologists was 0.988 and 0.988–0.919, respectively. The area under the PRC plot generated by the deep learning algorithm and radiologists was 0.973 and 0.618– 0.958, respectively. The sensitivity, specificity, PPV, and NPV of the proposed deep learning algorithm were 98.0, 98.1, 84.5, and 99.8%, respectively. There was no significant difference in the diagnosis of DDH by the algorithm and the radiologist with experience in pediatric radiology (p = 0.180). However, the proposed model showed higher sensitivity, specificity, and PPV, compared to the radiologist without experience in pediatric radiology (p < 0.001). @*Conclusion@#The proposed deep learning algorithm provided an accurate diagnosis of DDH on hip radiographs, which was comparable to the diagnosis by an experienced radiologist.

Comparing Initial Magnetic Resonance Imaging Findings to Differentiate between Krabbe Disease and Metachromatic Leukodystrophy in Children

Purpose@#To identify characteristic magnetic resonance imaging (MRI) features to differentiate between Krabbe disease and metachromatic leukodystrophy (MLD) in young children. @*Materials and Methods@#We collected all confirmed cases of Krabbe disease and MLD between October 2004 and September 2020 at Seoul National University Children's Hospital. Patients with initial MRI available were included. Their initial MRIs were retrospectively reviewed for the following: 1) presence of white matter signal abnormality involving the periventricular and deep white matter, subcortical white matter, internal capsule, brainstem, and cerebellum; 2) presence of volume decrease and signal alteration in the corpus callosum and thalamus; 3) presence of the tigroid sign; 4) presence of optic nerve hypertrophy; and 5) presence of enhancement or diffusion restriction. @*Results@#Eleven children with Krabbe disease and 12 children with MLD were included in this study. There was no significant difference in age or symptoms at onset.Periventricular and deep white matter signal alterations sparing the subcortical white matter were present in almost all patients of the two groups. More patients with Krabbe disease had T2 hyperintensities in the internal capsule and brainstem than patients with MLDs. In contrast, more patients with MLD had T2 hyperintensities in the splenium and genu of the corpus callosum. No patient with Krabbe disease showed T2 hyperintensity in the corpus callosal genu. A decrease in volume in the corpus callosum and thalamus was more frequently observed in patients with Krabbe disease than in those with MLD. Other MRI findings including the tigroid sign and optic nerve hypertrophy were not significantly different between the two groups. @*Conclusion@#Signal abnormalities in the internal capsule and brainstem, decreased thalamic volume, decreased splenial volume accompanied by signal changes, and absence of signal changes in the callosal genu portion were MRI findings suggestive of Krabbe disease rather than MLD based on initial MRI. Other MRI findings such as the tigroid sign could not help differentiate between these two diseases.

Inflammatory responses of C57BL/6NKorl mice to dextran sulfate sodium-induced colitis: comparison between three C57BL/6N sub-strains / 한국실험동물학회지

Background@#Inflammatory bowel disease (IBD), including both Crohn’s disease and ulcerative colitis, are chronic human diseases that are challenging to cure and are often unable to be resolved. The inbred mouse strain C57BL/ 6 N has been used in investigations of IBD as an experimental animal model. The purpose of the current study was to compare the inflammatory responsiveness of C57BL/6NKorl mice, a sub-strain recently established by the National Institute of Food and Drug Safety Evaluation (NIFDS), with those of C57BL/6 N mice from two different sources using a dextran sulfate sodium (DSS)-induced colitis model. @*Results@#Male mice (8 weeks old) were administered DSS (0, 1, 2, or 3%) in drinking water for 7 days. DSS significantly decreased body weight and colon length and increased the colon weight-to-length ratio. Moreover, severe colitisrelated clinical signs including diarrhea and rectal bleeding were observed beginning on day 4 in mice administered DSS at a concentration of 3%. DSS led to edema, epithelial layer disruption, inflammatory cell infiltration, and cytokine induction (tumor necrosis factor-α, interleukin-6, and interleukin-1β) in the colon tissues. However, no significant differences in DSS-promoted abnormal symptoms or their severity were found between the three sub-strains. @*Conclusions@#These results indicate that C57BL/6NKorl mice responded to DSS-induced colitis similar to the generally used C57BL6/N mice, thus this newly developed mouse sub-strain provides a useful animal model of IBD.

Monitoring Cerebral Perfusion Changes Using Arterial Spin-Labeling Perfusion MRI after Indirect Revascularization in Children with Moyamoya Disease

Objective@#To assess the role of arterial spin-labeling (ASL) perfusion MRI in identifying cerebral perfusion changes after indirect revascularization in children with moyamoya disease. @*Materials and Methods@#We included pre- and postoperative perfusion MRI data of 30 children with moyamoya disease (13 boys and 17 girls; mean age ± standard deviation, 6.3 ± 3.0 years) who underwent indirect revascularization between June 2016 and August 2017. Relative cerebral blood flow (rCBF) and qualitative perfusion scores for arterial transit time (ATT) effects were evaluated in the middle cerebral artery (MCA) territory on ASL perfusion MRI. The rCBF and relative time-topeak (rTTP) values were also measured using dynamic susceptibility contrast (DSC) perfusion MRI. Each perfusion change on ASL and DSC perfusion MRI was analyzed using the paired t test. We analyzed the correlation between perfusion changes on ASL and DSC images using Spearman’s correlation coefficient. @*Results@#The ASL rCBF values improved at both the ganglionic and supraganglionic levels of the MCA territory after surgery (p = 0.040 and p = 0.003, respectively). The ATT perfusion scores also improved at both levels (p < 0.001 and p < 0.001, respectively). The rCBF and rTTP values on DSC MRI showed significant improvement at both levels of the MCA territory of the operated side (all p < 0.05). There was no significant correlation between the improvements in rCBF values on the two perfusion images (r = 0.195, p = 0.303); however, there was a correlation between the change in perfusion scores on ASL and rTTP on DSC MRI (r = 0.701, p < 0.001). @*Conclusion@#Recognizing the effects of ATT on ASL perfusion MRI may help monitor cerebral perfusion changes and complement quantitative rCBF assessment using ASL perfusion MRI in patients with moyamoya disease after indirect revascularization.

Diagnostic Performance of a New Convolutional Neural Network Algorithm for Detecting Developmental Dysplasia of the Hip on Anteroposterior Radiographs

Objective@#To evaluate the diagnostic performance of a deep learning algorithm for the automated detection of developmental dysplasia of the hip (DDH) on anteroposterior (AP) radiographs. @*Materials and Methods@#Of 2601 hip AP radiographs, 5076 cropped unilateral hip joint images were used to construct a dataset that was further divided into training (80%), validation (10%), or test sets (10%). Three radiologists were asked to label the hip images as normal or DDH. To investigate the diagnostic performance of the deep learning algorithm, we calculated the receiver operating characteristics (ROC), precision-recall curve (PRC) plots, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) and compared them with the performance of radiologists with different levels of experience. @*Results@#The area under the ROC plot generated by the deep learning algorithm and radiologists was 0.988 and 0.988–0.919, respectively. The area under the PRC plot generated by the deep learning algorithm and radiologists was 0.973 and 0.618– 0.958, respectively. The sensitivity, specificity, PPV, and NPV of the proposed deep learning algorithm were 98.0, 98.1, 84.5, and 99.8%, respectively. There was no significant difference in the diagnosis of DDH by the algorithm and the radiologist with experience in pediatric radiology (p = 0.180). However, the proposed model showed higher sensitivity, specificity, and PPV, compared to the radiologist without experience in pediatric radiology (p < 0.001). @*Conclusion@#The proposed deep learning algorithm provided an accurate diagnosis of DDH on hip radiographs, which was comparable to the diagnosis by an experienced radiologist.

Comparing Initial Magnetic Resonance Imaging Findings to Differentiate between Krabbe Disease and Metachromatic Leukodystrophy in Children

Purpose@#To identify characteristic magnetic resonance imaging (MRI) features to differentiate between Krabbe disease and metachromatic leukodystrophy (MLD) in young children. @*Materials and Methods@#We collected all confirmed cases of Krabbe disease and MLD between October 2004 and September 2020 at Seoul National University Children's Hospital. Patients with initial MRI available were included. Their initial MRIs were retrospectively reviewed for the following: 1) presence of white matter signal abnormality involving the periventricular and deep white matter, subcortical white matter, internal capsule, brainstem, and cerebellum; 2) presence of volume decrease and signal alteration in the corpus callosum and thalamus; 3) presence of the tigroid sign; 4) presence of optic nerve hypertrophy; and 5) presence of enhancement or diffusion restriction. @*Results@#Eleven children with Krabbe disease and 12 children with MLD were included in this study. There was no significant difference in age or symptoms at onset.Periventricular and deep white matter signal alterations sparing the subcortical white matter were present in almost all patients of the two groups. More patients with Krabbe disease had T2 hyperintensities in the internal capsule and brainstem than patients with MLDs. In contrast, more patients with MLD had T2 hyperintensities in the splenium and genu of the corpus callosum. No patient with Krabbe disease showed T2 hyperintensity in the corpus callosal genu. A decrease in volume in the corpus callosum and thalamus was more frequently observed in patients with Krabbe disease than in those with MLD. Other MRI findings including the tigroid sign and optic nerve hypertrophy were not significantly different between the two groups. @*Conclusion@#Signal abnormalities in the internal capsule and brainstem, decreased thalamic volume, decreased splenial volume accompanied by signal changes, and absence of signal changes in the callosal genu portion were MRI findings suggestive of Krabbe disease rather than MLD based on initial MRI. Other MRI findings such as the tigroid sign could not help differentiate between these two diseases.

Contrast-enhanced voiding urosonography for the diagnosis of vesicoureteral reflux and intrarenal reflux: a comparison of diagnostic performance with fluoroscopic voiding cystourethrography

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.

Diplopia after Isolated Inferior Orbital Wall Fracture According to the Computed Tomography Findings

Purpose@#To investigate the postoperative changes in diplopia and evaluate factors that affect diplopia after isolated inferior orbital wall fracture. @*Methods@#A retrospective study was conducted on 200 patients who underwent reconstruction surgery for isolated inferior orbital wall fracture between March 2001 and December 2020. Diplopia was categorized into peripheral, central, and full-degree types based on the history and binocular single vision. The natural course of diplopia was analyzed pre-operatively and at 1 day, 6 weeks, and 6 months postoperatively. We analyzed the orbital computed tomography images to determine fracture size (small, medium, or large), fracture site (anterior, middle, posterior, or mixed), extraocular muscle (EOM) size (swelling or deformity), EOM site (incarcerated or prolapsed), and EOM tenting. @*Results@#Diplopia was seen in 144 out of 200 patients (72.0%) before surgery; peripheral diplopia was seen in 57 patients (39.6%), central diplopia in 46 (24.3%), and full-degree diplopia in 20 (13.9%). Pre-operative diplopia differed according to the pre-operative limitation of range of motion (LOM) (p < 0.001) and EOM site (p = 0.022). Pre-operative diplopia type differed according to the LOM direction (p = 0.018) and EOM size (p = 0.020). Diplopia persisted in 33 patients (16.5%) 6 months after the surgery. Recovery was faster in patients with peripheral diplopia compared to those with central or full-degree diplopia. @*Conclusions@#The presence of diplopia was associated with the pre-operative LOM and EOM site but the type of diplopia was associated with LOM direction and EOM size. These factors can be used to predict the progression of diplopia in patients with isolated inferior orbital wall fracture.

Clinical features and pulmonary function in children with Swyer-James-Macleod syndrome: A single center experience

Purpose@#Swyer-James-Macleod syndrome (SJMS) is a rare disorder characterized by the hyperlucency of a unilateral lung due to ipsilaterally arrested lung growth. No sufficient information on progressive lung function changes during growth is available in SJMS. @*Methods@#We retrospectively reviewed SJMS cases using a clinical database from January 2000 to August 2018. @*Results@#A total of 40 patients were enrolled in this study. The median age of the patients was 4.8 years (interquartile range [IQR], 2.7–9.0 years), boys (n = 21, 52.5%) were more affected, and left lungs (n = 27, 67.5%) were more involved. The interval between infection and diagnosis was 1.8 years (IQR, 0.7–6.4 years). Mycoplasma pneumoniae (n = 14) and adenovirus (n = 5) were most prevalent among the 21 subjects with presumably identifiable causes. Most of the 16 patients with available pulmonary function data presented moderate obstructive lung function (z-score, median; FEV 1: -4.320 [IQR, -5.955 to -3.400] and FVC: -2.600 [IQR, -3.590 to -1.570], respectively) with the more decreased median in forced expiratory flow between 25% and 75% of FVC (FEF 25%–75%) (z-score, -4.470; IQR, -5.330 to -3.170). Linear mixed effect models indicated that younger age at first infectious insult was significantly related to decreased FEV1 z-score (P = 0.041), and that the FEV 1/FVC and FEF 25%–75% z-scores were reduced by 0.082 (P = 0.005) and 0.069 (P ≤ 0.001) per year. @*Conclusion@#In children with SJMS, the initial infectious insult occurs in the early life with M. pneumoniae being the most common pathogen. Lung function may deteriorate with an obstructive pattern during growth. The impairment can progress slowly not in the FEV1, but in the FEV1/FVC or FEF25%–75%

An Electronic Health Record-Integrated Computerized Intravenous Insulin Infusion Protocol: Clinical Outcomes and in Silico Adjustment

BACKGROUND: We aimed to describe the outcome of a computerized intravenous insulin infusion (CII) protocol integrated to the electronic health record (EHR) system and to improve the CII protocol in silico using the EHR-based predictors of the outcome.METHODS: Clinical outcomes of the patients who underwent the CII protocol between July 2016 and February 2017 and their matched controls were evaluated. In the CII protocol group (n=91), multivariable binary logistic regression analysis models were used to determine the independent associates with a delayed response (taking ≥6.0 hours for entering a glucose range of 70 to 180 mg/dL). The CII protocol was adjusted in silico according to the EHR-based parameters obtained in the first 3 hours of CII.RESULTS: Use of the CII protocol was associated with fewer subjects with hypoglycemia alert values (P=0.003), earlier (P=0.002), and more stable (P=0.017) achievement of a glucose range of 70 to 180 mg/dL. Initial glucose level (P=0.001), change in glucose during the first 2 hours (P=0.026), and change in insulin infusion rate during the first 3 hours (P=0.029) were independently associated with delayed responses. Increasing the insulin infusion rate temporarily according to these parameters in silico significantly reduced delayed responses (P<0.0001) without hypoglycemia, especially in refractory patients.CONCLUSION: Our CII protocol enabled faster and more stable glycemic control than conventional care with minimized risk of hypoglycemia. An EHR-based adjustment was simulated to reduce delayed responses without increased incidence of hypoglycemia.