Purpose: With the revision of the Organ and Transplantation Act in 2018, the hand has become legal as an area of transplantable organs in Korea. In January 2021, the first hand allotransplantation since legalization was successfully performed, and we have performed a total of three successful hand transplantation since then. By comparing and incorporating our experiences, this study aimed to provide a comprehensive reconstructive solution for hand amputation in Korea. Materials and Methods: Recipients were selected through a structured preoperative evaluation, and hand transplantations were performed at the distal forearm level. Postoperatively, patients were treated with three-drug immunosuppressive regimen, and functional outcomes were monitored. Results: The hand transplantations were performed without intraoperative complications. All patients had partial skin necrosis and underwent additional surgical procedures in 2 months after transplantation. After additional operations, no further severe complications were observed. Also, patients developed acute rejection within 3 months of surgery, but all resolved within 2 weeks after steroid pulse therapy. Motor and sensory function improved dramatically, and patients were very satisfied with the appearance and function of their transplanted hands. Conclusion Hand transplantation is a viable reconstructive option, and patients have shown positive functional and psychological outcomes. Although this study has limitations, such as the small number of patients and short follow-up period, we should focus on continued recovery of hand function, and be careful not to develop side effects from immunosuppressive drugs. Through the present study, we will continue to strive for a bright future regarding hand transplantation in Korea.

Objectives: The purpose of this animal research was to compare bone regeneration in augmented rabbit maxillary sinuses treated with demineralized particulate human-tooth graft and anorganic bovine bone by immunohistochemical analysis. Materials and Methods: Piezoelectric bilateral sinus augmentation was performed in eight adult rabbits. In the control group, anorganic bovine was grafted in the maxillary sinus following elevation of the sinus membrane. In the experimental group, demineralized human particulate tooth bone was grafted in the sinus. Bone regeneration in augmented sinuses was evaluated by immunohistochemical analysis using various markers of osteoprogenitor cells. Results: The number of bromodeoxyuridine-labeled cells was significantly higher in the experimental group than in the control group at eight weeks. The immunoreactivity of proliferating-cell nuclear antigen was increased slightly in the experimental group relative to the control group at eight weeks. Other bone markers were expressed equally in the two groups. Conclusion In the rabbit maxillary sinus, higher osteoinduction was correlated with demineralized human particulate tooth bone grafting than with anorganic bovine grafting.

Objectives: The purpose of this animal research was to compare bone regeneration in augmented rabbit maxillary sinuses treated with demineralized particulate human-tooth graft and anorganic bovine bone by immunohistochemical analysis. Materials and Methods: Piezoelectric bilateral sinus augmentation was performed in eight adult rabbits. In the control group, anorganic bovine was grafted in the maxillary sinus following elevation of the sinus membrane. In the experimental group, demineralized human particulate tooth bone was grafted in the sinus. Bone regeneration in augmented sinuses was evaluated by immunohistochemical analysis using various markers of osteoprogenitor cells. Results: The number of bromodeoxyuridine-labeled cells was significantly higher in the experimental group than in the control group at eight weeks. The immunoreactivity of proliferating-cell nuclear antigen was increased slightly in the experimental group relative to the control group at eight weeks. Other bone markers were expressed equally in the two groups. Conclusion In the rabbit maxillary sinus, higher osteoinduction was correlated with demineralized human particulate tooth bone grafting than with anorganic bovine grafting.

PURPOSE: To evaluate the efficiency of the electronic foot function index (eFFI) through a prospective, random based, multi-institutional study. MATERIALS AND METHODS: The study included 227 patients ranging in age from 20 to 79 years, visited for surgery in different 15 institutes, and agreed to volunteer. The patients were assigned randomly into a paper-based evaluated group (n=113) and tablet-based evaluated group (n=114). The evaluation was done on the day of hospital admission and the method was changed on the second day of surgery and re-evaluated. PADAS 2.0 (https://www.proscore.kr) was used as an electronic evaluation program. RESULTS: There were no differences in age and sex in both groups. The intraclass correlation coefficient (ICC) evaluation revealed an eFFI ICC of 0.924, showing that both results were similar. The evaluation time was shorter in the tablet-based group than the paper-based group (paper vs tablet, 3.7±3.8 vs 2.3±1.3 minutes). Thirty-nine patients (17.2%) preferred to use paper and 131 patients (57.7%) preferred the tablet. Fifty-seven patients (25.1%) found both ways to be acceptable. CONCLUSION: eFFI through tablet devices appears to be more constant than the paper-based program. In addition, it required a shorter amount of time and the patients tended to prefer the tablet-based program. Overall, tablet and cloud system can be beneficial to a clinical study.
Academies and Institutes ; Ankle ; Clinical Study ; Foot ; Humans ; Methods ; Prospective Studies ; Volunteers

PURPOSE: The authors measured the anteversion of the femoral neck and acetabulum and the sum of the two values in normal Korean people by computed tomography. The authors examined the normal range of the values to analyze the difference in sex and sides as well as the relationship between the femoral neck and acetabular anteversion. MATERIALS AND METHODS: The authors measured the anteversion of the femoral neck and acetabulum in 118 normal Korean adult males and 114 females aged between 21 and 49 on both the right and left sides by computed tomography and calculated the sum of anteversion. The authors analyzed the mean and standard deviation, and investigated the sex differences and side differences, as well as the relationship between the acetabular anteversion and femoral neck anteversion statistically. RESULTS: The anteversion of the acetabulum in males was 15.3°±6.1° on the right side and 15.3°±6.6° on the left side. The anteversion of the femoral neck in males was 5.3°±7.6° on the right side and 1.5°±9.2° on the left side according to the Hernandez et al. method. The anteversion of acetabulum in females was 16.8°±5.4° on the right side and 16.3°±5.8° on the left side. The anteversion of femoral neck in females was 10.3°±8.2° on the right side and 7.9°±8.2° on the left side according to Hernandez et al. method. No difference in acetabular anteversion, and a significant difference in the femoral neck anteversion on both the right and left sides were observed between males and females. No difference of acetabular anteversion was observed between the right and left sides, but a significant difference in femoral neck anteversion was noted between the right and left sides measured by either the Hernandez et al. method or Weiner et al. method. The Pearson coefficient revealed no correlation between the femoral neck anteversion and acetabular anteversion. CONCLUSION: No difference in the acetabular anteversion was observed, but there was a significant difference in femoral neck anteversion between males and females. A significant difference in femoral neck anteversion was observed between the right and left sides. No correlation was noted between the anteversion of the femoral neck and acetabulum.
Acetabulum ; Adult ; Female ; Femur Neck ; Humans ; Male ; Methods ; Reference Values ; Sex Characteristics

PURPOSE: Mutations in the cyclin-dependent kinase-like 5 (CDKL5) gene are associated with epileptic encephalopathy and severe cognitive impairment. We aim to characterize the association between this gene and treatment efficacy. METHODS: We retrospectively analyzed 10 patients who were treated at Severance Children's Hospital for epileptic encephalopathy who were subsequently diagnosed with a CDKL5 mutation using next-generation sequencing. RESULTS: Electroencephalography (EEG) results showed generalized pattern abnormalities in 60% (6/10) of patients with CDKL5 mutations. We analyzed the effects of three treatments, namely antiepileptic drugs (AEDs), ketogenic diet (KD), and steroids. A more than 50% reduction in seizures was observed in 12% (1/8) of patients treated with clobazam. KD treatment proved ineffective in most cases. In addition, a more than 50% reduction in seizures was observed in 57% (4/7) of patients treated with steroids. EEG analysis of patients treated effectively with steroids revealed that 75% (3/4) showed hypsarrhythmia and 25% (1/4) showed focal epileptiform. CONCLUSION In this study, as in other studies, AEDs and KD did not effectively control seizures in most patients with a CDKL5 mutation. However, steroid therapy reduced the frequency of seizures in patients who also exhibited hypsarrhythmia. This suggests that steroid treatment is helpful in cases of hypsarrhythmia with CDKL5 mutations.

BACKGROUND AND OBJECTIVES: A risk prediction is needed even in the contemporary era of acute myocardial infarction (AMI). We sought to develop a risk scoring specific for patients with AMI being treated with guideline-adherent optimal therapies, including percutaneous coronary intervention and all 5 medications (aspirin, thienopyridine, β-blocker, angiotensin-converting enzyme inhibitor or angiotensin receptor blocker, and statin). METHODS: From registries, 12,174 AMI patients were evaluated. The primary outcome was 1-year all-cause death or AMI. The Korea Working Group in Myocardial Infarction (KorMI) system was compared with the Assessment of Pexelizumab in Acute Myocardial Infarction (APEX AMI), Controlled Abciximab and Device Investigation to Lower Late Angioplasty Complications (CADILLAC), and Global Registry of Acute Coronary Events scores (GRACE) models. RESULTS: Ten predictors were identified: left ventricular dysfunction (hazard ratio [HR], 2.3), bare-metal stent (HR, 2.0), Killip class ≥II (HR, 1.9), renal insufficiency (HR, 1.8), previous stroke (HR, 1.6), regional wall-motion- score >20 on echocardiography (HR, 1.5), body mass index ≤24 kg/m2 (HR, 1.4), age ≥70 years (HR, 1.4), prior coronary heart disease (HR, 1.4), and diabetes (HR, 1.4). Compared with the previous models, the KorMI system had good discrimination (time-dependent C statistic, 0.759) and showed reasonable goodness-of-fit by Hosmer-Lemeshow test (p=0.84). Moreover, the continuous-net reclassification improvement varied from −27.3% to −19.1%, the integrated discrimination index varied from −2.1% to −0.9%, and the median improvement in risk score was from −1.0% to −0.4%. CONCLUSIONS: The KorMI system would be a useful tool for predicting outcomes in survivors treated with guideline-adherent optimal therapies after AMI.
Angioplasty ; Angiotensins ; Body Mass Index ; Coronary Disease ; Discrimination (Psychology) ; Drug Therapy ; Echocardiography ; Humans ; Korea ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Registries ; Renal Insufficiency ; Stents ; Stroke ; Survivors ; Ventricular Dysfunction, Left

KBG syndrome is a rare neurodevelopmental disorder characterized by intellectual disability, skeletal anomalies, short stature, craniofacial dysmorphism, and macrodontia. ANKRD11 gene mutation and 16q24.3 microdeletion have been reported to cause KBG syndrome. Here, we report two patients with ANKRD11 mutations who initially presented with neurologic symptoms such as developmental delay and seizures. Patient 1 was a 23-month-old boy who presented with a global developmental delay. Language delay was the most dominant feature. He had hypertelorism, hearing impairment, and behavior problems characterized as hyperactivity. A c.1903_1907delAAACA (p.Lys635GInfsTer26) mutation in ANKRD11 was identified with diagnostic exome sequencing. Patient 2 was a 14-month-old boy with developmental delay and seizure. He also had atrial septum defect, and ventricular septal defect. Generalized tonic seizures began at the age of 8 months. Electroencephalography showed generalized sharp and slow wave pattern. Seizures did not respond to antiepileptic drugs. A loss of function mutation c.5350_5351delTC (p.ser1784HisfsTer12) in ANKRD11 was identified with diagnostic exome sequencing. In both cases, characteristic features of KBG syndrome such as short stature or macrodontia, were absent, and they visited the hospital due to neurological symptoms. These findings suggest that more patients with mild phenotypes of KBG syndrome are being recognized with advances in diagnostic exome sequencing genetic technologies.
Anticonvulsants ; Atrial Septum ; Developmental Disabilities ; Early Diagnosis* ; Electroencephalography ; Exome* ; Hearing Loss ; Heart Septal Defects, Ventricular ; Humans ; Hypertelorism ; Infant ; Intellectual Disability ; Language Development Disorders ; Male ; Neurodevelopmental Disorders ; Neurologic Manifestations ; Phenotype ; Seizures

BACKGROUND AND OBJECTIVES: A risk prediction is needed even in the contemporary era of acute myocardial infarction (AMI). We sought to develop a risk scoring specific for patients with AMI being treated with guideline-adherent optimal therapies, including percutaneous coronary intervention and all 5 medications (aspirin, thienopyridine, β-blocker, angiotensin-converting enzyme inhibitor or angiotensin receptor blocker, and statin). METHODS: From registries, 12,174 AMI patients were evaluated. The primary outcome was 1-year all-cause death or AMI. The Korea Working Group in Myocardial Infarction (KorMI) system was compared with the Assessment of Pexelizumab in Acute Myocardial Infarction (APEX AMI), Controlled Abciximab and Device Investigation to Lower Late Angioplasty Complications (CADILLAC), and Global Registry of Acute Coronary Events scores (GRACE) models. RESULTS: Ten predictors were identified: left ventricular dysfunction (hazard ratio [HR], 2.3), bare-metal stent (HR, 2.0), Killip class ≥II (HR, 1.9), renal insufficiency (HR, 1.8), previous stroke (HR, 1.6), regional wall-motion- score >20 on echocardiography (HR, 1.5), body mass index ≤24 kg/m2 (HR, 1.4), age ≥70 years (HR, 1.4), prior coronary heart disease (HR, 1.4), and diabetes (HR, 1.4). Compared with the previous models, the KorMI system had good discrimination (time-dependent C statistic, 0.759) and showed reasonable goodness-of-fit by Hosmer-Lemeshow test (p=0.84). Moreover, the continuous-net reclassification improvement varied from −27.3% to −19.1%, the integrated discrimination index varied from −2.1% to −0.9%, and the median improvement in risk score was from −1.0% to −0.4%. CONCLUSIONS The KorMI system would be a useful tool for predicting outcomes in survivors treated with guideline-adherent optimal therapies after AMI.

Morel-Lavallée is a rare lesion caused by post-traumatic soft tissue injury. It usually occurs around the greater trochanter, and it occurs very rarely in the lumbar region. It is often difficult to be diagnosed in the emergency room. Delayed diagnosis may result in the need for open surgery. The authors report a patient with extensive multiple Morel-Lavallée lesions in the thoracolumbar, buttock, and thigh after trauma and provide a literature review.
Buttocks ; Delayed Diagnosis ; Emergency Service, Hospital ; Femur ; Humans ; Lumbosacral Region ; Soft Tissue Injuries ; Thigh