Patients with Beckwith-Wiedemann syndrome (BWS) are predisposed to developing embryonal tumors, with hepatoblastoma being the most common type. Our patient showed hemihypertrophy, macroglossia, and paternal uniparental disomy in chromosome 11 and was diagnosed with BWS. When the patient was 9 months old, a 2.5×1.5 cm oval hypoechoic exophytic mass was detected in the inferior tip of his right liver. Preoperative imaging identified it as hepatoblastoma; however, histologic, immunohistochemistry, and electron microscopic findings were compatible with adrenal cortical neoplasm with uncertain malignant potential. The origin of the adrenal tissue seemed to be heterotopic. Here, we describe for the first time an adrenal cortical neoplasm with uncertain malignant potential arising in the heterotopic adrenal cortex located in the liver of a patient with BWS.
Adrenal Cortex ; Adrenal Gland Neoplasms ; Beckwith-Wiedemann Syndrome ; Chromosomes, Human, Pair 11 ; Hepatoblastoma ; Humans ; Immunohistochemistry ; Liver ; Macroglossia ; Uniparental Disomy

A previously healthy, 3-year-old boy presented to the emergency department with an afebrile focal motor seizure. He was found crying and having a seizure 30 minutes earlier. During this seizure, he was jerking his head and right extremities. Subsequent magnetic resonance imaging showed acute infarction in the bilateral frontal lobes, chiefly in the left. After hospitalization, conventional angiography demonstrated bilateral stenosis of the distal internal carotid arteries with development of lenticulostriate collaterals, which confirmed the diagnosis of moyamoya disease. It is vital to recognize focal motor seizures and situations related to hyperventilation in children with a seizure, which imply a structural lesion and a provoked cerebral ischemia in preexisting moyamoya disease, respectively.
Angiography ; Brain Ischemia ; Carotid Artery, Internal ; Child ; Child, Preschool ; Constriction, Pathologic ; Crying ; Diagnosis ; Emergencies ; Emergency Service, Hospital ; Extremities ; Frontal Lobe ; Head ; Hospitalization ; Humans ; Hyperventilation ; Infarction ; Ischemia ; Magnetic Resonance Imaging ; Male ; Moyamoya Disease ; Seizures ; Stroke ; Vasoconstriction

Purpose: This study evaluated the accuracy of attenuation imaging (ATI) for the assessment of hepatic steatosis in pediatric patients, in comparison with the FibroScan vibration-controlled transient elastography controlled attenuation parameter (CAP). Methods: Consecutive pediatric patients referred for evaluation of obesity who underwent both ATI and FibroScan between February 2020 and September 2021 were included. The correlation between attenuation coefficient (AC) and CAP values was assessed using the Spearman test. The AC cutoff value for discriminating hepatic steatosis corresponding to a CAP value of 241 dB/m was calculated. Multivariable linear regression analysis was performed to estimate the strength of the association between AC and CAP. The diagnostic accuracy of AC cutoffs was estimated using the imperfect gold-standard methodology based on a two-level Bayesian latent class model. Results: Seventy patients (median age, 12.5 years; interquartile range, 11.0 to 14.0 years; male:female, 58:12) were included. AC and CAP showed a moderate-to-good correlation (ρ =0.646, P<0.001). Multivariable regression analysis affirmed the significant association between AC and CAP (P<0.001). The correlation was not evident in patients with a body mass index ≥30 kg/m2 (ρ=-0.202, P=0.551). Linear regression revealed that an AC cutoff of 0.66 dB/cm/MHz corresponded to a CAP of 241 dB/m (sensitivity, 0.93; 95% confidence interval [CI], 0.85 to 0.98 and specificity, 0.87; 95% CI, 0.56 to 1.00). Conclusion ATI showed an acceptable correlation with CAP values in a pediatric population, especially in patients with a body mass index <30 kg/m2. An AC cutoff of 0.66 dB/cm/MHz, corresponding to a CAP of 241 dB/m, can accurately diagnose hepatic steatosis.

Background: and Purpose: Recent quantitative neuroimaging studies of childhood absence epilepsy (CAE) have identified various structural abnormalities that might be involved in the onset of absence seizure and associated cognitive and behavioral functions. However, the neuroanatomical alterations specific to CAE remain unclear, and so this study investigated the regional alterations of brain structures associated with newly diagnosed CAE. Methods: Surface and volumetric magnetic resonance imaging data of patients with newly diagnosed CAE (n=18) and age-matched healthy controls (n=18) were analyzed using FreeSurfer software. A group comparison using analysis of covariance was performed with significance criteria of p<0.05 andp<0.01 in global and regional analyses, respectively. Results: Compared with control subjects, the patients with CAE had smaller total and regional volumes of cortical gray-matter (GM) in the right rostral middle frontal, right lateral orbitofrontal, and left rostral middle frontal regions, as well as in the right precentral, right superior, middle, left middle, and inferior temporal gyri. The cortex in the right posterior cingulate gyrus and left medial occipital region was significantly thicker in patients with CAE than in controls. Conclusions Patients with CAE showed a reduced bilateral frontotemporal cortical GM volume and an increased posterior medial cortical thickness, which are associated with the default mode network. These structural changes can be suggested as the neural basis of the absence seizures and neuropsychiatric comorbidities in CAE.

From January 1998 to December 2002, 3,259 cases of uterine myoma were treated at the department of Obstetrics and Gynecology, Chunbuk National University Hospital. A clinico-stastical study of uterine myoma was perfomed to analyse the clinical characteristics. The results were as follows. 1. The incidence of uterine myoma was 9.8%. 2. The most frequent age group was 40 to 49 years, and the mean age was 44.6 years. 3. The average parity was 2.29, the infertility was 163 cases (5.0%), while the primary infertility, 2.4%, the secondary, 2.6% respectively. 4. The most frequent chief complaint was pain which was observed in 2,648 cases (81.2%), abnormal bleeding in 1,775 cases (53.8%). dizziness in 270 cases (8.3%). 5. The corporeal myomas were observed in 2,879 cases (95.9%). Intramural type was observed in 1,687 cases (58.2%), subserous in 529 cases (18.2%), submucous in 191 cases (6.5%), mixed type in 483 cases (17.0%). 6. The mean value of preoperative hemoglobin was 11.1 gm/dL, and the anemia (Hb<10.0 gm/dL) was observed in 481 cases (11.7%). Transfusion was necessary in 215 cases (6.5%). 7. The mean weight of the uterine myoma operated was 335.0 gm. 8. The secondary change of myoma was found in 54 cases (1.7%) and hyaline degeneration was the most common (0.7%). 9. The most common associated condition was chronic cervicitis, which was observed in 784 cases (24.1%). 10. The gynecologic surgery were performed in 1,456 cases (44.7%), medical therapy in 25 cases (0.8%), observation in 1,792 cases (55.0%). 11. Total abdominal hysterectomy was performed in 607 cases (41.7%), total abdominal hysterectomy with unilateral adnexectomy in 115 cases (7.9%), total abdominal hysterectomy with both adnexectomy in 164 cases (11.3%), subtotal hysterectomy in 304 cases (20.9%), myomectomy in 153 cases (10.5%), total laparoscopic hysterectomy in 103 cases (7.0%), laparoscopy assisted vaginal hysterectomy in 8 cases (0.5%), diagnostic laparoscopy in 2 cases (0.1%) respectively. 12. The postoperative complication were found in 113 cases (3.5%) and the wound infection was the most common (1.6%). 13. The mean period of hospitalization was 8 days, and the period less than 10 days in 1,177 cases (80.9%).
Anemia ; Dizziness ; Female ; Gynecologic Surgical Procedures ; Gynecology ; Hemorrhage ; Hospitalization ; Humans ; Hyalin ; Hysterectomy ; Hysterectomy, Vaginal ; Incidence ; Infertility ; Laparoscopy ; Leiomyoma* ; Myoma ; Obstetrics ; Parity ; Postoperative Complications ; Uterine Cervicitis ; Wound Infection

OBJECTIVE: To assess the clinical efficacy of alternative techniques for biliary stricture cannulation in patients undergoing living donor liver transplantation (LDLT), after cannulation failure with a conventional (0.035-inch guidewire) technique. SUBJECTS AND METHODS: Of 293 patients with biliary strictures after LDLT, 19 (6%) patients, 11 men and 8 women of mean age 48.5 years, had the failed cannulation of the stricture by conventional techniques. Recannulation was attempted by using two alternative methods, namely a micro-catheter set via percutaneous access and a snare (rendezvous) technique using percutaneous and endoscopic approaches. RESULTS: Strictures were successfully cannulated in 16 (84%) of the 19 patients. A microcatheter set was used in 12 and a snare technique in four patients. Stricture cannulation failed in the remaining three patients, who finally underwent surgical revision. CONCLUSION: Most technical failures using a conventional technique for biliary stricture cannulation after LDLT can be overcome by using a microcatheter set or a snare (rendezvous) technique.
Adult ; Bile Ducts/*surgery ; Catheterization/*methods ; Constriction, Pathologic ; Female ; Fluoroscopy ; Humans ; *Liver Transplantation ; *Living Donors ; Male ; Middle Aged ; Reoperation ; Treatment Outcome

OBJECTIVE: To determine the rate with which diagnostic test accuracy studies that are published in a general radiology journal adhere to the Standards for Reporting of Diagnostic Accuracy Studies (STARD) 2015, and to explore the relationship between adherence rate and citation rate while avoiding confounding by journal factors. MATERIALS AND METHODS: All eligible diagnostic test accuracy studies that were published in the Korean Journal of Radiology in 2011-2015 were identified. Five reviewers assessed each article for yes/no compliance with 27 of the 30 STARD 2015 checklist items (items 28, 29, and 30 were excluded). The total STARD score (number of fulfilled STARD items) was calculated. The score of the 15 STARD items that related directly to the Quality Assessment of Diagnostic Accuracy Studies (QUADAS)-2 was also calculated. The number of times each article was cited (as indicated by the Web of Science) after publication until March 2016 and the article exposure time (time in months between publication and March 2016) were extracted. RESULTS: Sixty-three articles were analyzed. The mean (range) total and QUADAS-2-related STARD scores were 20.0 (14.5-25) and 11.4 (7-15), respectively. The mean citation number was 4 (0-21). Citation number did not associate significantly with either STARD score after accounting for exposure time (total score: correlation coefficient = 0.154, p = 0.232; QUADAS-2-related score: correlation coefficient = 0.143, p = 0.266). CONCLUSION: The degree of adherence to STARD 2015 was moderate for this journal, indicating that there is room for improvement. When adjusted for exposure time, the degree of adherence did not affect the citation rate.
Checklist ; Compliance ; Diagnostic Tests, Routine* ; Publications

Congenital isolated hydronephrosis encompasses a spectrum of physiologic states that spontaneously resolve and pathologic obstruction that necessitates surgical intervention. Distinguishing patients whose condition will resolve, those who will require stringent follow-up, and those who will eventually need surgical intervention present a challenge to clinicians, particularly because no unified guidelines for assessment and follow-up have been established. The recognition of the natural course and prognosis of hydronephrosis and a comprehensive understanding of the currently proposed consensus guidelines may aid in multidisciplinary treatment and in providing proper counseling to caregivers. In this review, we aimed to summarize the literature on the grading systems and management strategies for congenital isolated hydronephrosis.

Purpose: To evaluate the applicability of Greulich-Pyle (GP) standards to bone age (BA) assessment in healthy Korean children using manual and deep learning-based methods. Materials and Methods: We collected 485 hand radiographs of healthy children aged 2–17 years (262 boys) between 2008 and 2017. Based on GP method, BA was assessed manually by two radiologists and automatically by two deep learning-based BA assessment (DLBAA), which estimated GP-assigned (original model) and optimal (modified model) BAs. Estimated BA was compared to chronological age (CA) using intraclass correlation (ICC), Bland-Altman analysis, linear regression, mean absolute error, and root mean square error. The proportion of children showing a difference >12 months between the estimated BA and CA was calculated. Results: CA and all estimated BA showed excellent agreement (ICC ≥0.978, p<0.001) and significant positive linear correlations (R2 ≥0.935, p<0.001). The estimated BA of all methods showed systematic bias and tended to be lower than CA in younger patients, and higher than CA in older patients (regression slopes ≤-0.11, p<0.001). The mean absolute error of radiologist 1, radiologist 2, original, and modified DLBAA models were 13.09, 13.12, 11.52, and 11.31 months, respectively. The difference between estimated BA and CA was >12 months in 44.3%, 44.5%, 39.2%, and 36.1% for radiologist 1, radiologist 2, original, and modified DLBAA models, respectively. Conclusion Contemporary healthy Korean children showed different rates of skeletal development than GP standard-BA, and systemic bias should be considered when determining children’s skeletal maturation.

Purpose: The aim of this study was to validate the postnatal urinary tract dilation (UTD) classification system by correlating it with the need for surgical intervention. Methods: Young infants who underwent ultrasound (US) examinations for prenatal hydronephrosis were retrospectively identified. The kidney units (KUs; right, left, or bilateral) were graded from UTD P0 (very low risk) to P3 (high risk) based on seven US criteria from the UTD system. Surgery-free survival curves were constructed using the Kaplan-Meier method. Univariable and multivariable Cox proportional-hazards regression analysis clustered by patients was performed. Interobserver agreement was analyzed using the weighted kappa coefficient. Results: In total, 504 KUs from 336 patients (mean age, 18.3±15.9 days; range, 1 to 94 days; males, n=276) were included, with a median follow-up of 24.2 months. Fifty-eight KUs underwent surgical intervention. Significant differences were observed among the Kaplan-Meier curves stratified into UTD groups (P<0.001). The presence of anterior-posterior renal pelvic diameter ≥15 mm (hazard ratio [HR], 8.602; 95% confidence interval [CI], 1.558 to 43.065), peripheral calyceal dilation (HR, 8.190; 95% CI, 1.558 to 43.065), ureteral dilation (HR, 2.619; 95% CI, 1.274 to 5.380), parenchymal thickness abnormality (HR, 3.371; 95% CI, 1.574 to 7.223), bladder abnormality (HR, 12.209; 95% CI, 3.616 to 41.225) were significantly associated with the occurrence of surgery. The interobserver agreement was moderate to almost perfect agreement for US features (κ=0.564-0.898) and substantial for final UTD grades (κ=0.716). Conclusion The UTD classification system is reliable and appropriately stratifies the risk of surgical intervention.