Favorable outcomes with durable chimerism after hematopoietic cell transplantation using busulfan and fludarabine-based reduced-intensity conditioning for pediatric patients with hemophagocytic lymphohistiocytosis

Background@#The incorporation of a reduced-intensity conditioning (RIC) regimen in hematopoietic cell transplantation (HCT) for patients with hemophagocytic lymphohistiocytosis (HLH) has decreased early mortality but is associated with a high rate of mixed chimerism and graft failure. Here, we present a successful single-center experience using busulfan and a fludarabine-based RIC regimen for the treatment of HLH. @*Methods@#The medical records of pediatric patients with HLH who underwent HCT using a busulfan/fludarabine-based RIC regimen between January 2008 and December 2017 were reviewed retrospectively. @*Results@#Nine patients received HCT with a busulfan/fludarabine-based RIC regimen. Three patients had primary HLH, and the other six patients had secondary HLH with multiple reactivations. All three patients with primary HLH had UNC13D mutations. All patients achieved neutrophil and platelet engraftment at a median of 11 days (range, 10‒21) and 19 days (range, 13‒32), and all eight evaluable patients had sustained complete donor chimerism at the last follow-up. Two patients (22%) experienced grade 2 acute graft-versus-host disease (GVHD). Two patients (22%) developed chronic GVHD, and one died from chronic GVHD. One patient (11%) experienced reactivation 4 months after HCT from a syngeneic donor and died of the disease. The 8-year overall survival and event-free survival rates were 78%. No early treatment-related mortality within 100 days after HCT was observed. @*Conclusion@#Our experience suggests that a busulfan/fludarabine-based RIC regimen is a viable option for pediatric patients with HLH who require HCT.

Growth Pattern of the Neo-Aorta after Arterial Switch Operation during Childhood

Background and Objectives@#Neo-aortic root dilatation (ARD) and annular dilatation (AAD) tend to develop after arterial switch operation (ASO). However, the trend of neo-aortic growth has not been well established. This paper aims to identify this trend, its associated factors, and predictors of neo-aortic dilatation after ASO. @*Methods@#We analyzed the growth trend of the neo-aortic root, annulus, and sinotubular junction (STJ) z-scores using random coefficients model and the risk factors affecting neoaortic dilatation in 163 patients who underwent ASO from 2006 to 2015. @*Results@#Among 163 patients, 41 had a ventricular septal defect, and 11 had Taussig-Bing (TB) anomaly. The median follow-up duration was 6.61 years. The increased in the neo-aortic root z-score was different between the trapdoor and non-trapdoor coronary artery transfer techniques (0.149/year, p<0.001 vs. 0.311/year, p<0.001). Moreover, the neo-aortic annulus and STJ z-score significantly increased over time after ASO (0.067/year, p<0.001; 0.309/ year, p<0.001). Pulmonary artery banding (PAB) was rather a negative affecting factor. The probabilities of freedom from ARD, AAD, and neo-aortic STJ dilatation at 10 years after ASO were 33.4%, 53.9%, and 65.4%. Neo- aortic regurgitation within 1 year was the predictor of ARD, AAD, and neo-aortic STJ dilatation. TB anomaly, PAB, and native pulmonary sinus z-score were other predictors for ARD. @*Conclusion@#The growth of neo-aortic root, annulus, and STJ after ASO was greater than somatic growth during childhood. The coronary artery transfer technique affected the growth pattern of the neo-aortic root.

Relationship of Bone Mineral Density and Knee Osteoarthritis (Kellgren-Lawrence Grade):Fifth Korea National Health and Nutrition Examination Survey

Background@#Osteoarthritis (OA) and osteoporosis (OP) are the 2 most common bone disorders associated with aging. We can simply assume that older patients have a higher incidence of OA and OP with more severity. Although several papers have conducted studies on the relationship between OA and OP, none of them has demonstrated a conclusive link. In this study, we used radiological knee OA and bone mineral density (BMD; T-score of the total hip and lumbar spine) to analyze the incidence of OA and OP in a large population. We aimed to determine the relationship between OA and OP and investigate the associated risk factors. @*Methods@#This cross-sectional study used data extracted from the 2010–2012 Korea National Health and Nutrition Examination Survey. We evaluated a total of 4,250 participants aged ≥ 50 years who underwent knee radiography and dual-energy X-ray absorptiometry and their laboratory results. The relationship between radiological knee OA and BMD was assessed. The generalized linear model was used to evaluate the relationship between BMD and Kellgren-Lawrence (KL) grade. @*Results@#The higher KL grade was associated with older age, higher body mass index (BMI), female sex, and lower hemoglobin level (p 0.05). After adjusting for confounding factors (age, BMI, diabetes, hypertension, smoking, and alcohol consumption), the average T-scores of total hip and lumbar spine were the highest in the mild OA group with KL grade 2 (–0.22 ± 1.08 and –0.89 ± 1.46, respectively,p < 0.001). The average T-scores of the total hip and lumbar spine significantly decreased as OA progressed from moderate (KL grade 3; –0.49 ± 1.05 and –1.33 ± 1.38, respectively, p < 0.001) to severe (KL grade 4; –0.73 ± 1.13 and –1.74 ± 1.75, respectively, p < 0.001). T-scores of the moderate-to-severe OA group were significantly lower than those of the non-OA group (KL grades 0 and 1, p < 0.001). @*Conclusions@#Compared with the non-OA group, BMD (T-scores of the total hip and lumbar spine) was higher in the mild OA group and lower in the moderate-to-severe OA group.

Growth Pattern of the Neo-Aorta after Arterial Switch Operation during Childhood

Background and Objectives@#Neo-aortic root dilatation (ARD) and annular dilatation (AAD) tend to develop after arterial switch operation (ASO). However, the trend of neo-aortic growth has not been well established. This paper aims to identify this trend, its associated factors, and predictors of neo-aortic dilatation after ASO. @*Methods@#We analyzed the growth trend of the neo-aortic root, annulus, and sinotubular junction (STJ) z-scores using random coefficients model and the risk factors affecting neoaortic dilatation in 163 patients who underwent ASO from 2006 to 2015. @*Results@#Among 163 patients, 41 had a ventricular septal defect, and 11 had Taussig-Bing (TB) anomaly. The median follow-up duration was 6.61 years. The increased in the neo-aortic root z-score was different between the trapdoor and non-trapdoor coronary artery transfer techniques (0.149/year, p<0.001 vs. 0.311/year, p<0.001). Moreover, the neo-aortic annulus and STJ z-score significantly increased over time after ASO (0.067/year, p<0.001; 0.309/ year, p<0.001). Pulmonary artery banding (PAB) was rather a negative affecting factor. The probabilities of freedom from ARD, AAD, and neo-aortic STJ dilatation at 10 years after ASO were 33.4%, 53.9%, and 65.4%. Neo- aortic regurgitation within 1 year was the predictor of ARD, AAD, and neo-aortic STJ dilatation. TB anomaly, PAB, and native pulmonary sinus z-score were other predictors for ARD. @*Conclusion@#The growth of neo-aortic root, annulus, and STJ after ASO was greater than somatic growth during childhood. The coronary artery transfer technique affected the growth pattern of the neo-aortic root.

Trampoline-related injuries in Korea

The growing popularity of trampolines in Korea has resulted in a significant increase in the number of injuries associated with their use. However, little research has been conducted into trampoline-related injuries in Asian countries, including Korea and the incidence and characteristics of trampoline-related injuries have rarely been reported. In a review of the very few studies on these injuries in Korea, the mean age at injury was younger than has been reported in previous studies from other countries, and 46% of patients experienced fracture. Most injuries (82%) occurred in commercial facilities. In Korea, the safety management of trampolines is insufficient. Hence, safety guidelines for the use of trampolines should be recommended by relevant medical professionals.

Pediatric Trampoline-Related Injuries in a Nationwide Registry in South Korea, 2011 to 2016

PURPOSE: Trampoline-related injuries are steadily increasing. To our knowledge, there have been very few studies on trampoline injuries in Asia. The purpose of this study is to report the characteristics of pediatric trampoline injuries in Korea. MATERIALS AND METHODS: We conducted a nationwide retrospective cohort study. Data were collected from prospective nationwide databases (Emergency Department-based Injury In-depth Surveillance databases of the Korea Centers for Disease Control and Prevention) for patients who visited emergency departments (EDs) after injuries during 2011–2016. RESULTS: Of 263712 patients between 0 and 17 years of age, 2799 patients with trampoline injuries visited EDs. The median age of the patients was 5 years (interquartile range, 3–8 years), and 63% of the patients were under 6 years old. Of the patients, 1526 (54.2%) were male. Seventy-six percent of injuries occurred at trampoline parks. Trampoline injuries and trampoline park injuries have increased steadily, while ages at injury have gradually decreased year by year (p < 0.001). Injury locations included the lower extremity (47%), head and face (24%), and upper extremity (24%). A fracture was sustained by 886 (31.7%) patients. The distal humerus and proximal tibia were the most common fracture sites (34% and 23%, respectively). Fractures occurred more commonly in trampoline parks than in homes (33.7% vs. 21.1%, p < 0.001). CONCLUSION: In Korea, pediatric trampoline injuries and trampoline park injuries have tended to increase, while ages at injury have tended to decrease. Policies to prevent trampoline injuries are needed.

Traumatic Separation of Bipartite Patella Underlying Gout

Gouty arthritis is a common crystal arthropathy, but gout tophus in the bipartite patella is a rare condition. This report presented a traumatic separation of bipartite patellar fragment caused by mild trauma in a patient with comorbid gout. When a patient with bipartite patella and underlying gouty arthritis complains of pain after trauma, clinical suspicion is needed about fragment separation of the bipartite patella.

Trampoline-related injuries in Korea

The growing popularity of trampolines in Korea has resulted in a significant increase in the number of injuries associated with their use. However, little research has been conducted into trampoline-related injuries in Asian countries, including Korea and the incidence and characteristics of trampoline-related injuries have rarely been reported. In a review of the very few studies on these injuries in Korea, the mean age at injury was younger than has been reported in previous studies from other countries, and 46% of patients experienced fracture. Most injuries (82%) occurred in commercial facilities. In Korea, the safety management of trampolines is insufficient. Hence, safety guidelines for the use of trampolines should be recommended by relevant medical professionals.

Quantitative Measurement of Muscle Atrophy and Fat Infiltration of the Supraspinatus Muscle Using Ultrasonography After Arthroscopic Rotator Cuff Repair

OBJECTIVE: To investigate the utility of ultrasonography to objectively examine morphological changes (i.e., muscle atrophy and fatty infiltration) of the supraspinatus muscle. METHODS: Thirty-four patients were prospectively enrolled in this study. The degrees of muscle atrophy and fat infiltration were measured using ultrasonography 3–4 months after arthroscopic supraspinatus tendon repair. Shoulder function (i.e., shoulder active range of motion, visual analogue scale, and constant score) was examined. Using the symmetricity of the muscles in the human body, the degrees of morphological changes of the supraspinatus muscle were quantitatively measured. The associations between the morphological changes of the supraspinatus muscle and shoulder function were identified. RESULTS: There were statistically significant differences in the cross-sectional area (CSA) and echogenicity between the surgery and non-surgery sides (p < 0.001). The CSA ratio, which represents the degree of muscle atrophy, was associated with shoulder forward flexion, external rotation, and constant score; however, the echogenicity ratio, which represents the degree of fat infiltration, was not associated with shoulder function after surgery. CONCLUSION: This study demonstrated that shoulder function could be predicted by evaluating the morphological changes of the supraspinatus muscle using ultrasonography and that objective evaluation is possible through quantitative measurement using the symmetricity of the human body.

Anatomic Repair Including Hemi-Mustard in Congenitally Corrected Transposition of the Great Arteries: What We know and Still Need to Know

No abstract available.

Intramedullary Nailing for Pathological Fractures of the Proximal Humerus

BACKGROUND: Endoprosthetic reconstruction is widely applied for pathological fractures of the proximal humerus; however, functional impairment is usually unsatisfactory. The aims of the current study are to evaluate the reliability of interlocking intramedullary (IM) nailing with cement augmentation as a fixation method in proximal humeral lesions and to assess functional outcomes. METHODS: We reviewed 32 patients with pathological fractures of the proximal humerus who underwent interlocking IM nailing and cement augmentation. Functional scores and pain relief were assessed as outcomes. RESULTS: The mean follow-up period was 14.2 months. The mean Musculoskeletal Tumor Society functional score and Karnofsky performance status scale score were 27.7 and 75.6, respectively. Improvement of pain assessed using the visual analogue scale was 6.2 on average. Thirty-one patients (97%) experienced no pain after surgery. The mean ranges of forward flexion and abduction were 115° and 112.6°, respectively. All patients achieved stability and had no local recurrence without failure of fixation until the last follow-up. CONCLUSIONS: Proximal interlocking IM nailing with cement augmentation appears to be a reliable treatment option for pathological or impending fractures of the proximal humerus in selected patients with metastatic tumors, even with extensive bone destruction.

Impact of postoperative duration of Aspirin use on longevity of bioprosthetic pulmonary valve in patients who underwent congenital heart disease repair / 소아과

PURPOSE: Generally, aspirin is used as a protective agent against thrombogenic phenomenon after pulmonary valve replacement (PVR) using a bioprosthetic valve. However, the appropriate duration of aspirin use is unclear. We analyzed the impact of postoperative duration of aspirin use on the longevity of bioprosthetic pulmonary valves in patients who underwent repair for congenital heart diseases. METHODS: We retrospectively reviewed the clinical data of 137 patients who underwent PVR using a bioprosthetic valve between January 2000 and December 2003. Among these patients, 89 were included in our study and divided into groups I (≤12 months) and II (>12 months) according to duration of aspirin use. We analyzed echocardiographic data from 9 to 11 years after PVR. Pulmonary vale stenosis and regurgitation were classified as mild, moderate, or severe. RESULTS: The 89 patients consisted of 53 males and 36 females. Their mean age was 14.3±8.9 years (range, 2.6–48 years) and body weight was 37.6±14.7 kg (range, 14–72 kg). The postoperative duration of aspirin use was 7.3±2.9 months in group I and 32.8±28.4 months in group II. However, no significant difference in sex ratio, age, body weight, type of bioprosthetic valve, and number of early redo-PVRs. In the comparison of echocardiographic data about 10 years later, no significant difference in pulmonary valve function was found. The overall freedom rate from redo-PVR at 10 years showed no significant difference (P=0.498). CONCLUSION: Our results indicated no benefit from long-term aspirin medication (>6 months) in patients who underwent PVR with a bioprosthetic valve.

Cardiopulmonary Bypass Priming Using Autologous Cord Blood in Neonatal Congenital Cardiac Surgery

BACKGROUND AND OBJECTIVES: A blood transfusion is almost inevitable in neonatal cardiac surgery. This study aimed to assess the feasibility of using autologous cord blood for a cardiopulmonary bypass (CPB) priming as an alternative to an allo-transfusion in neonatal cardiac surgery. SUBJECTS AND METHODS: From January 2012 to December 2014, cord blood had been collected during delivery after informed consent and was stored immediately into a blood bank. Eight neonatal patients had their own cord blood used for CPB priming during cardiac surgery. RESULTS: All patients underwent surgery for their complex congenital heart disease. The median age and body weight at surgery was 11 days (from 0 to 21 days) and 3.2 kg (from 2.2 to 3.7 kg). The median amount and hematocrit of collected cord blood was 72.5 mL (from 43 to 105 mL) and 48.7% (from 32.0 to 51.2%). The median preoperative hematocrit of neonates was 36.5% (from 31.0 to 45.0%); the median volume of CPB priming was 130 mL (From 120 to 140 mL). Seven out of eight patients did not need an allo-transfusion in CPB priming and only one neonate used 20 mL of packed red blood cells in CPB priming to obtain the target hematocrit. CONCLUSION: Autologous cord blood can be used for CPB priming as alternative to packed red blood cells in neonatal congenital cardiac surgery in order to reduce allo-transfusion.

Surgical Management of Mitral Regurgitation in Patients with Marfan Syndrome during Infancy and Early Childhood

BACKGROUND: Mitral regurgitation is one of the leading causes of cardiovascular morbidity in pediatric patients with Marfan syndrome. The purpose of this study was to contribute to determining the appropriate surgical strategy for these patients. METHODS: From January 1992 to May 2013, six patients with Marfan syndrome underwent surgery for mitral regurgitation in infancy or early childhood. RESULTS: The median age at the time of surgery was 47 months (range, 3 to 140 months) and the median follow-up period was 3.6 years (range, 1.3 to 15.5 years). Mitral valve repair was performed in two patients and four patients underwent mitral valve replacement with a mechanical prosthesis. There was one reoperation requiring valve replacement for aggravated mitral regurgitation two months after repair. The four patients who underwent mitral valve replacement did not experience any complications related to the prosthetic valve. One late death occurred due to progressive emphysema and tricuspid regurgitation. CONCLUSION: Although repair can be an option for some patients, it may not be durable in infantile-onset Marfan syndrome patients who require surgical management during infancy or childhood. Mitral valve replacement is a feasible treatment option for these patients.

Incidence and Treatment Pattern of Extremity Soft Tissue Sarcoma in Korea, 2009-2011: A Nationwide Study Based on the Health Insurance Review and Assessment Service Database / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: We conducted a nationwide study to assess the incidence and treatment patterns of extremity soft tissue sarcoma (STS) in South Korea. MATERIALS AND METHODS: The nationwide incidence and treatment patterns of extremity STS were assessed using two nationwide databases, the Korea National Cancer Incidence (KNCI) database and the Health Insurance Review and Assessment Service (HIRA) database. RESULTS: A total of 1,236 patients were newly diagnosed with extremity STS during the 3-year study period, from 2009 to 2011. The annual incidence of extremity STS in the Korean population was approximately 0.9 per 100,000 people with a male bias that increased with age and was especially pronounced amongst individuals aged > 80 years. Approximately 7% of patients did not receive any treatment, and surgical excision was performed for 85% of those who were treated. CONCLUSION: This is the first nationwide study of the incidence and treatment patterns of extremity STS in Korea using two national databases (KNCI and HIRA), which include the entire Korean population. The results of this study may be useful for future planning and management of STS, at the national level.

Favorable outcome of allogeneic hematopoietic stem cell transplantation followed by post-transplant treatment with imatinib in children with Philadelphia chromosome-positive acute lymphoblastic leukemia

BACKGROUND: Allogeneic hematopoietic stem cell transplantation (HSCT) is the preferred curative therapy for children with Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL). We evaluated the treatment outcomes of children with Ph+ ALL who underwent allogeneic HSCT. METHODS: Fifteen children diagnosed with Ph+ ALL in Asan Medical Center Children's Hospital between 1998 and 2012 were retrospectively analyzed. RESULTS: Of 521 children diagnosed with ALL during the study period, 15 had a Philadelphia chromosome. Among these 15 patients, 13 attained complete remission (CR) following induction chemotherapy, and two died of intracerebral hemorrhage during leukapheresis and induction chemotherapy, respectively. Of the 13 patients who attained CR, 12 received allogeneic HSCT, mainly from unrelated donors. Of the 12 patients who received HSCT, one died of a transplant-related cause, one died of relapse after HSCT, and 10 remain in continuous CR. Of the 10 patients who remained in CR longer than six months after HSCT, seven received post-HSCT imatinib. For all 15 patients, the 5-year overall survival, event-free survival, and cumulative incidence of relapse were 60.0%, 48.6%, and 38.8%, respectively, with a median follow-up of 70 months. For the HSCT group, the 5-year overall survival, event-free survival, and cumulative incidence of relapse were 80.2%, 72.9%, and 29.3%, respectively, with a median follow-up of 100 months. CONCLUSION: Allogeneic HSCT cures a significant proportion of Ph+ ALL patients. Because the use of imatinib appears to be a promising approach, strategies that include tyrosine kinase inhibitors before and after HSCT require further evaluation.

Distinct Clinical Characteristics of Unplanned Excision in Synovial Sarcoma

BACKGROUND: We aimed to describe the clinical characteristics and outcomes of unplanned excisions of synovial sarcomas. METHODS: In total, 90 patients with synovial sarcomas in the extremities were retrospectively reviewed. Patients were divided into unplanned excision (n = 38) and planned excision (n = 52) groups. The average follow-up period was 6 years. The clinicopathological characteristics and oncologic outcomes were compared. RESULTS: The unplanned excision group showed longer duration of symptoms before diagnosis (p = 0.023), smaller lesion dimensions (p = 0.001), superficial location (p = 0.049), and predilection in the upper extremities (p = 0.037). Synovial sarcomas were most commonly misdiagnosed as neurogenic tumors (56%) in the upper extremities or as cystic masses (47%) in the lower extremities. Oncological outcomes, including disease-specific survival, metastasis-free survival, or local recurrence were not significantly different between the 2 groups (p = 0.159, p = 0.444, and p = 0.335, respectively). Repeated unplanned excision (p = 0.012) and delayed re-excision (p = 0.038) were significant risk factors for local recurrence in the unplanned excision group. CONCLUSIONS: Synovial sarcomas treated with unplanned excision had distinct characteristics. These findings are important for developing diagnostic and therapeutic strategies for synovial sarcoma.

Successful Surgical Treatment of Cardiac Complication of Graves Disease

Cardiac complications such as arrhythmia and heart failure are common in Graves disease. Early detection and proper treatment of hyperthyroidism are important because cardiac complications are reported to be reversible if the thyroid function is normalized by medical treatment. We report here a case of cardiac complication of Graves disease that was too late to reverse with medical treatment and required surgical treatment.

Intercalary Allograft Reconstruction after Malignant Tumor Resection around the Knee / 대한정형외과학회잡지

PURPOSE: We aimed to assess the treatment outcomes and factors affecting bone union of intercalary allograft reconstruction after primary malignant bone tumor resection around the knee. MATERIALS AND METHODS: Twenty-one patients who underwent intercalary allograft reconstruction after resection of a malignant tumor of femur or tibia were retrospectively reviewed. The average follow-up period was 46.4 months. Location of the tumor was as follows: femur in 12 cases and tibia in 9. Osteosarcoma was the most common tumor (12 cases). Intercalary allograft was internally fixed with an intramedullary (IM) nail in 3 cases, with a plate in 12 cases and with an IM nail combined with a plate in 6 cases. The survival of the grafts and functional outcomes were evaluated. Factors affecting bone union and complications were assessed. RESULTS: All allografts survived without removal. The average Musculoskeletal Tumor Society functional score was 27. The mean length of the allograft was 16.7 cm and bony union took 10.9 months, in average. Nonunion occurred in 6 cases: at the diaphyseal side in 3, and the remaining 3 at the metaphyseal side. The graft length was shorter than average in all the diaphyseal side nonunion cases and longer than average in all the metaphyseal side nonunion cases. All cases of nonunion obtained bone union after additional operations (autologous bone graft alone: 1, bone graft with hardware change: 5). CONCLUSION: Survival and functional outcomes of intercalary allograft were satisfactory. Long allograft showed a tendency of fracture or nonunion at the metaphyseal side. Nonunion could be managed with additional operation without allograft removal.

Occurrence of Intestinal Pseudo-obstruction in a Brainstem Hemorrhage Patient

Intestinal pseudo-obstruction is a massive colonic dilation with signs and symptoms of colonic obstruction, but without a mechanical cause. A 49-year-old female patient complained of nausea, vomiting, and abdominal distension 1 month after a massive brainstem hemorrhage. No improvement was seen with conservative treatments. An extended-length rectal tube was inserted to perform glycerin enema. In addition, bethanechol (35 mg per day) was administered to stimulate colonic motility. The patient's condition gradually improved over a 2-month period without any surgical intervention. Extended length rectal tube enema and bethanechol can be used to improve intestinal pseudo-obstruction in stroke patients.