Purpose: Biliary atresia (BA) is a rare but progressive cholangiopathy and the leading cause of pediatric liver transplantation worldwide. The Korean Association of Pediatric Surgeons (KAPS) has conducted three national surveys (2001, 2011, and 2023) to assess long-term trends in the diagnosis, treatment, and outcomes of BA. This study provides a comparative analysis of the 2nd and 3rd national surveys, with reference to selected findings from the 1st survey. Methods: This study included 453 patients from the 3rd national survey (2011–2021) and 435 patients from the 2nd survey (2001–2010), all of whom underwent Kasai portoenterostomy. Data were collected via electronic case report forms from pediatric surgical centers nationwide. Comparisons were made regarding demographics, clinical features, diagnostic patterns, operative details, follow-up outcomes, and survival. Kaplan–Meier analysis was used to evaluate long-term survival. Results: The mean number of BA patients per year remained stable between surveys (43.5 in the 2nd, 41.18 in the 3rd), though centralization of care increased, with 61.5% of cases managed by two major institutions in the 3rd survey. The median age at surgery decreased, and the use of preoperative imaging (especially magnetic resonance cholangiopancreatography) increased. The 10-year native liver survival rate declined from 59.8% to 53.7%, while overall 10-year survival improved slightly (92.9% to 93.2%). Postoperative complications, such as cholangitis and liver failure, persisted but were better categorized. The 3rd survey also reported improved mortality (4.9%) and reduced follow-up loss (11.5%) compared to the 2nd survey. Conclusion While overall survival after Kasai operation has remained high and even improved, native liver survival has slightly declined. The findings reflect earlier diagnosis, more consistent diagnostic imaging, and increasing centralization of care. These trends underscore the importance of long-term nationwide data collection in guiding future strategies for BA management in Korea.

Purpose: This study aims to investigate and compare the incidence, demographic characteristics, clinical manifestations, preoperative diagnostic methods, anatomical classifications, associated anomalies, operative treatments, and postoperative outcomes of patients with intestinal atresia treated by the members of the Korean Association of Pediatric Surgeons (KAPS) through three nationwide surveys. Methods: KAPS conducted 3 national surveys in 1998, 2010, and 2024 to examine the patients diagnosed with intestinal atresia. In preparation for the survey, we developed a customized case registration form to obtain data on patient sex, birth weight, gestational age, clinical manifestations, preoperative diagnostic methods, anatomical types, associated anomalies, operative treatments, and postoperative outcomes. Authorized KAPS members completed the case registration form. Results: The first, second, and third national surveys included 218, 222, and 236 individuals diagnosed with intestinal atresia, respectively. The male-to-female ratios were 1.5:1, 1.1:1, and 1.1:1, respectively. The first, second, and third national surveys revealed that 34.3%, 43.3%, and 53.4% of patients were born before 37 weeks of gestation, respectively. Additionally, 28.7%, 32.0%, and 40.7% of patients had a birth weight under 2,500 g. In the third national survey, duodenoduodenostomy was the most common procedure, performed in 70 out of 82 patients diagnosed with duodenal atresia. Resection and anastomosis were the main surgical procedures conducted in 47 out of 54 cases of jejunal atresia and 74 out of 92 cases of ileal atresia. The mortality rates in the first, second, and third national surveys were 13.8%, 3.6%, and 1.3% respectively, with the lowest rate observed in the third national survey. Conclusion These national surveys offer valuable insights into the current state of intestinal atresia, including specific surgical interventions and postoperative outcomes in South Korea. For pediatric surgeons aiming to enhance their understanding of intestinal atresia and its treatment options, these surveys could be an indispensable resource and guide.

Colorectal cancer is the third most common cancer in Korea and the third leading cause of death from cancer. Treatment outcomes for colon cancer are steadily improving due to national health screening programs with advances in diagnostic methods, surgical techniques, and therapeutic agents.. The Korea Colon Cancer Multidisciplinary (KCCM) Committee intends to provide professionals who treat colon cancer with the most up-to-date, evidence-based practice guidelines to improve outcomes and help them make decisions that reflect their patients’ values and preferences. These guidelines have been established by consensus reached by the KCCM Guideline Committee based on a systematic literature review and evidence synthesis and by considering the national health insurance system in real clinical practice settings. Each recommendation is presented with a recommendation strength and level of evidence based on the consensus of the committee.

Nephrogenic diabetes insipidus (NDI) can cause nonobstructive hydronephrosis. Congenital NDI (CNDI) is caused by a genetic mutation. This case report presents a 12-year-old girl who was incidentally diagnosed with nonobstructive hydronephrosis due to NDI caused by AQP2 gene mutation after being evaluated for microscopic hematuria found on routine health examination at school. The patient's medical and family history was unremarkable, and she complained of nocturia only at the time of the clinic visit. Bilateral hydronephrosis on abdominal ultrasonography prompted a water deprivation test, leading to diagnosis of NDI. Genetic study confirmed p.Asn (AAC)123Ser (AGC) in exon 2 of the AQP2 gene. Polyuria and hydronephrosis improved following arginine-vasopressin therapy. CNDI responsive to treatment should be considered as a possible cause of nonobstructive hydroureter.
Ambulatory Care ; Child ; Diabetes Insipidus, Nephrogenic* ; Diagnosis ; Exons ; Female ; Hematuria ; Humans ; Hydronephrosis* ; Nocturia ; Polyuria ; Ultrasonography ; Water Deprivation

OBJECTIVE: The purpose of this study is to investigate whether acoustic radiation force impulse (ARFI) elastography with ARFI quantification and ARFI 2-dimensional (2D) imaging is useful for differentiating hepatic hemangiomas from malignant hepatic tumors. MATERIALS AND METHODS: One-hundred-and-one tumors in 74 patients were included in this study: 28 hemangiomas, 26 hepatocellular carcinomas (HCCs), three cholangiocarcinomas (CCCs), 20 colon cancer metastases and 24 other metastases. B-mode ultrasound, ARFI 2D imaging, and ARFI quantification were performed in all tumors. Shear wave velocities (SWVs) of the tumors and the adjacent liver and their SWV differences were compared among the tumor groups. The ARFI 2D images were compared with B-mode images regarding the stiffness, conspicuity and size of the tumors. RESULTS: The mean SWV of the hemangiomas was significantly lower than the malignant hepatic tumor groups: hemangiomas, 1.80 +/- 0.57 m/sec; HCCs, 2.66 +/- 0.94 m/sec; CCCs, 3.27 +/- 0.64 m/sec; colon cancer metastases, 3.70 +/- 0.61 m/sec; and other metastases, 2.82 +/- 0.96 m/sec (p < 0.05). The area under the receiver operating characteristics curve of SWV for differentiating hemangiomas from malignant tumors was 0.86, with a sensitivity of 96.4% and a specificity of 65.8% at a cut-off value of 2.73 m/sec (p < 0.05). In the ARFI 2D images, the malignant tumors except HCCs were stiffer and more conspicuous as compared with the hemangiomas (p < 0.05). CONCLUSION: ARFI elastography with ARFI quantification and ARFI 2D imaging may be useful for differentiating hepatic hemangiomas from malignant hepatic tumors.
Adult ; Aged ; Carcinoma, Hepatocellular/*ultrasonography ; Diagnosis, Differential ; Elasticity Imaging Techniques/*methods ; Female ; Hemangioma/*ultrasonography ; Humans ; Liver/*ultrasonography ; Liver Neoplasms/*ultrasonography ; Male ; Middle Aged ; ROC Curve ; Reproducibility of Results

OBJECTIVE: To investigate the additional diagnostic value of color mapping of the hepatic arterial enhancement fraction (AEF) for detecting recurrent or residual hepatocellular carcinoma (HCC) in patients treated with transcatheter arterial chemoembolization (TACE). MATERIALS AND METHODS: Seventy-six patients with 126 HCCs, all of whom had undergone previous TACE, and subsequently, underwent follow-up multiphasic liver CT scans, were included in this study. Quantitative color maps of the AEF of the whole liver were created, by using prototype software with non-rigid registration. The AEF was defined as the ratio of the attenuation increment during the arterial phase to the attenuation increment during the portal phase. Two radiologists independently analyzed the two image sets at a two-week interval, i.e., the multiphasic CT image set and the second image set of the AEF color maps and the CT images. The additional diagnostic value of the AEF color mapping was determined, by the use of the jackknife-alternative free-response receiver-operating-characteristic analysis. The sensitivity and positive predictive values for detecting HCCs of each image set were also evaluated and compared. RESULTS: The reader-averaged figures of merit were 0.699 on the initial interpretation of the MDCT image set, and 0.831 on the second interpretation of the combined image set; the difference between the two interpretations was significant (p value < 0.001). The mean sensitivity for residual or recurrent HCC detection increased from 62.7% on the initial analysis to 82.1% on the second analysis using the AEF color maps (p value < 0.001). The mean positive predictive value for HCC detection was 74.5% on the initial analysis using MDCT, and 71.6% on the second analysis using AEF color mapping. CONCLUSION: Quantitative color mapping of the hepatic AEF may have the possibility to increase the diagnostic performance of MDCT for the detection of recurrent or residual HCC without the potential risk of radiation-related hazards.
Aged ; Carcinoma, Hepatocellular/pathology/*radiography ; *Chemoembolization, Therapeutic ; Female ; Humans ; Liver Cirrhosis/*complications/radiography ; Liver Neoplasms/pathology/*radiography ; Male ; Middle Aged ; Neoplasm Recurrence, Local/pathology/*radiography ; Predictive Value of Tests ; ROC Curve ; Radiographic Image Interpretation, Computer-Assisted ; Retrospective Studies ; Sensitivity and Specificity ; Software ; *Tomography, X-Ray Computed

OBJECTIVE: To investigate the training effects on balance and gait ability using balance control trainer combined with partial weight-bearing system in chronic hemiplegic stroke patients. METHOD: A prospective crossover clinical trial was designed. The subjects consisted of 16 chronic hemiplegic stroke patients. All patients had a stroke more than six months. In addition to conventional physical therapy (PT), 8 patients in group A were trained with the balance control trainer for 30 min/day, 5 day/week, for first 2 weeks and then received only conventional physical therapy for 2 weeks. The other 8 patients in group B received only conventional PT for first 2 weeks and then were trained with the balance control trainer for 30 min/day, 5 day/week, for next 2 weeks, with additional conventional PT. We evaluated with clinical tests including functional ambulation categories (FAC), Berg balance scale (BBS), 6 min walking distance (6mWT), timed up and go (TUG), Korean-modified barthel Index (K-MBI) and muscle strengthening of knee extensor (MMTknee) before training, 2 weeks and 4 weeks after training in those patients. RESULTS: After training, subjects in experimental period (2weeks period of conventional PT+Balance control trainer in group A&B) showed more improvement than those in control period (2 weeks period of only conventional PT in group A&B) in FAC, BBS, 6mWT, TUG, K-MBI (p<0.05). CONCLUSION: We think the balance control trainer combined with the partial weight-bearing system can be a useful tool for improving balance and gait ability in chronic hemiplegic stroke patients.
Gait ; Humans ; Knee ; Muscles ; Prospective Studies ; Stroke ; Walking ; Weight-Bearing

F-18 FDG PET is a metabolic imaging modality that is efficacious in staging and assessment of treatment response for variety of lymphomas. We report usefulness of F-18 FDG PET/CT in evaluating severity of the disease and response to therapy in a patient with subcutaneous panniculitis- like T-cell lymphoma (SPTCL). Here we describe a case of SPTCL in 24-year-old man who had wide spread firm and tender nodular lesions with increased F-18 FDG uptake. After chemotherapy follow up F-18 FDG PET/CT image shows disseminated malignancy and then the patient died with hemophagocytic syndrome. This report suggests that F-18 FDG PET/CT may be useful in determining disease activity at the time of initial diagnosis, after treatment, and evaluating a suspected outcome of SPTCL.
Follow-Up Studies ; Humans ; Lymphohistiocytosis, Hemophagocytic ; Lymphoma ; Lymphoma, T-Cell ; Panniculitis ; Young Adult

PURPOSE: Elbow osteoarthritis with ulnar neuropathy was treated by a modified posteromedial approach, for decompression/transposition of the ulnar nerve and simultaneously with an Outerbridge-Kashiwagi procedure. The clinical result with these operative techniques is reported. MATERIALS AND METHODS: Average age of the patients was 51, which including 9 male and 2 female. There were 8 manual workers and one each of a clerk, a merchant and a house wife. The ulnar neuropathy was evaluated by the McGowan grading; one grade I, 4 grade II and 6 grade III. RESULTS: The ulnar nerve symptoms were relieved in all patients; 3 McGowan grade I, 5 grade II and 3 grade III, postoperatively. The pain subsided in all patients, with the exception of in one. The average range of motion was improved from 22.5-124degrees to 11.5-128.5degrees. CONCLUSION: A modified posteromedial approach is an effective method for both ulnar nerve decompression and the OK procedure, and provides an effective functional outcome.
Decompression ; Elbow* ; Female ; Humans ; Male ; Osteoarthritis* ; Range of Motion, Articular ; Spouses ; Ulnar Nerve ; Ulnar Neuropathies*

PURPOSE: We analyzed the comparative results of limb alignment after total knee arthroplasty using conventional method and image-free navigation system. MATERIALS AND METHODS: Total knee arthroplasties were performed in sixty-four patients(74 knees) with conventional method(37 cases)(Group A) and Orthopilot 4.0 image-free navigation system(37 cases)(Group B). We measured the femoro-tibial angle, femoral and tibial component angle in coronal and sagittal plane using the weight bearing whole leg radiograph taken 1-month postoperatively. RESULTS: Mechanical femoro-tibial angle was 1.4 degrees of varus in conventional group (Group A) and 0.8 degrees of varus in the navigation group (Group B) on average. Femoral and tibial component angle in the coronal plane was mean 1.8 degrees of varus and 0.7 degrees of varus in group A, 0.3 degrees of valgus and 0.5 degrees of varus in group B on average. Average femoral and tibial component angle in sagittal plane was 0.3 degrees of flexion and 0.8 degrees of anterior tilt in group A, 0.7 degrees of flexion and 1.7 degrees of anterior tilt in group B. There were no statistically significant differences between two groups in the femoro-tibial angle, femoral and tibial component angle in coronal and sagittal plane. But the ranges of deviation from desired angle were smaller in Group B than Group A. Statistically significant different results of the limb alignment (femoro-tibial angle and femoral component angle in coronal plane) were observed in 5 cases with severely bowed femoral shaft in group B (average femoro-tibial angle 0.9 degrees and femoral component angle in coronal plane 0.9 degrees varus) than group A (average femoro-tibial angle 3.5 degrees and femoral component angle in coronal plane 3.9 degrees varus). CONCLUSION: Accuracy of limb alignment in total knee arthroplasty using navigation system was not statistically significant difference than using the conventional method, but had better results in the deviation of mechanical femoro-tibial angle and femoral component angle in coronal plane than the conventional method, especially in severely bowed femoral shaft.
Arthroplasty* ; Extremities* ; Knee* ; Leg ; Weight-Bearing