• Journal of the Korean Orthopaedic Association
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• v.53 no.6
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• pp.505-512
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• 2018

Purpose: Many reconstruction methods have been attempted after an en-bloc resection of the proximal humerus. In particular, the introduction of reverse shoulder arthroplasty (RSA) has made a breakthrough in the functional recovery of the shoulder. Nevertheless, RSA has limitations when the humeral bone stock loss is significant. In addition, it is unclear if RSA is effective in patients showing failure with non-operative treatment of a proximal humeral tumor. Materials and Methods: A reconstruction was performed using an overlapping allograft-RSA composite for 11 patients with a failed proximal humeral construct. Delayed RSA was performed on 6 patients with failed non-operative treatment. The pre- and postoperative Musculoskeletal Tumor Society (MSTS) score and the complications were addressed. Results: Overlapping allograft-RSA composite afforded a stable construct in 11 failed proximal humeral reconstructions and the patient's chief complaints were resolved. The mean time to the union of overlapped allograft-host junction was 5.5 months. Average preoperative MSTS score of 20.3 point increased to 25.7 point, postoperatively. Four of the six patients who had RSA within 4 years from the index operation showed arm elevation of more than $90^{\circ}$ whereas the remaining 5 patients showed some disability. The complications include one case each of dislocation and aseptic infection, which were resolved by changing the polyethylene liner and scar revision, respectively. None of the 6 patients who underwent delayed RSA after the failure of non-operative treatment showed arm elevation more than $90^{\circ}$. Conclusion: An overlapping allograft-RSA composite is a simple and reliable reconstructive modality in patients with massive bone loss. In patients with metastatic cancer necessitating a surgical resection at presentation, early conversion to RSA is recommended to secure functional recovery.

• The Journal of the Korean bone and joint tumor society
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• v.7 no.4
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• pp.139-143
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• 2001

Purpose : Limb salvage for osteosarcoma of proximal tibia is challenging problem due to difficulties in mobilizing or retracting the main neurovascular structure, inadequate soft tissue coverage, and unsolved problem of patellar tendon reattachment to endoprosthesis. The authors analyzed the functional result of limb salvage using tumor prosthesis with medial gastrocnemius rotation plasty for osteosarcama of the proximal tibia. Materials and Methods : Eleven patients with histologically proven osteosarcoma of the proximal tibia, treated with adjuvant and neoadjuvant chemotherapy and limb salvage operation with tumor prosthesis between January 1992 and December 1998 at our Medical Center, were selected. There were 6 male and 5 female. Age ranged from 15 years to 23.7 years with an average of 23.7 years. Follow-up period ranged from 1 year to 4.5 years with an average of 2.5 years. The final functional result was evaluated using the method by ISOLS, 1993. The factors include pain, functional activities, emotional acceptance, use of external supports, walking ability and gait. Each of the factors has been scored from 0 to 5 depending on the appropriate description or data. The rating score is determined by dividing the individual factor scores into the total score and indicates percentage of normal function. Results : The overall functional result ranged from 53,3% to 86.7% with an average of 68.3% of normal function. In details, the averages were 82.5% for pain, 62.5% for functional activities, 67.5% for emotional acceptance, 77.5% for use of external supports, 62.5% for walking ability, and 57.5% for gait. The average range of motion of the knee joint was $5^{\circ}$ extension and $85^{\circ}$ flexion. Five patients have extension lag ranged from $5^{\circ}$ to $15^{\circ}$ with an average of $10^{\circ}$. Two patients suffered postoperative infection. One was treated with antibiotics injection only, but the other needed removal of the prosthesis and knee fusion. Both of them showed unsatisfactory result. C o n c l u s i o n : The overall functional result after limb salvage using tumor prosthesis with medial gastrocnemius rotational flap for osteosarcoma of the proximal tibia was relatively satisfactory in case of no postoperative infection. The patients were less satisfactory in functional activities, emotional acceptance and gait than pain, use of external supports due to limitation of motion and extension lag. More aggressive postoperative physical therapy and protection with brace for 6~9 months as well as surgical technique is mandatory for more satisfactory result.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.812-819
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• 2008

Purpose : Vitamin D plays a key role in bone mineralization of the skeleton and vitamin D deficiency can lead to rickets. It is well known that vitamin D deficiency is common in breast fed infants. Of these patients, clinically, some have no signs of rickets, but laboratory and radiographic findings are diagnostic for vitamin D deficiency rickets (subclinical vitamin D deficiency rickets). The purpose of this study is to clarify current causes and ways to prevent this disease. Methods : We reviewed the clinical and laboratory characteristics of children who were incidentally diagnosed as subclinical rickets during treatment of other disease such as pneumonia, gastroenteritis, urinary tract infection at Eulji Hospital, Seoul, Korea from March, 2003 to July 2007. Results : Eight patients (six boys and two girls) were diagnosed with subclinical vitamin D deficiency rickets. The mean age of the patients was $12.6{\pm}5.8months$, and they were diagnosed from January to July. The associated diseases were pneumonia, urinary tract infection, acute gastroenteritis, and iron deficiency anemia. All patients were breast-fed. Two showed growth failure. The mean serum alkaline phosphatase was $1995.8{\pm}739.5IU/L$, the mean calcium count was $9.5{\pm}0.6mg/dL$, and the mean phosphorus content was $3.6{\pm}1.5mg/dL$. The mean intact parathyroid hormone was $214.8{\pm}155.9pg/mL$ (reference range, 9-65), the mean 1,25-dihydroxyvitamin D was $82.4{\pm}49.3pg/mL$ (reference range, 2070), and the mean 25-hydroxyvitamin D was $29.6{\pm}10.6ng/mL$ (reference range, 1030). A radiographic examination showed cupping, fraying, and flaring of metaphyses in all patients. Six patients were administered calcitriol (400 IU/day) for three months. A consequent radiographic and laboratory examination showed improvement. The first two patients were initially diagnosed with metaphyseal dysplasia, without the detection of vitamin D deficiency and they spontaneously improved without vitamin D supplements. However, two years later, they showed mild scoliosis and metaphyseal dysplasia, respectively. Conclusion : Breast-feeding without supplementation involves high risk of vitamin D deficiency. Some infants may also develop rickets; therefore, such groups should be considered for vitamin D supplementation.

• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.173-180
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• 2006

Purpose : Cytomegalovirus(CMV) infection still remains as a major cause of morbidity and mortality after stem cell transplantation. In this study, we analyzed the results of antigenemia-guided preemptive therapy among children with allogeneic hematopoietic stem cell transplantation to determine the incidence and risk factors associated with CMV antigenemia, and evaluated the efficacy of the CMV antigenemia based preemptive therapy. Methods : We enrolled 213 pediatric patients following allogeneic hematopoietic stem cell transplantation(HSCT), at the Catholic HSCT center between October 1998 and December 2003. Pre-emptive ganciclovir was started when more than 5 CMV Ag-positive cells were detected in matched sibling HSCT, and when any Ag-positive cells were seen in unrelated allogenic HSCT. Results : CMV antigenemia was observed in 88(41.3 percent) of 213 patients on median day 28(day 11-99). In univariated analysis, use of unrelated donors(other than siblings), age of recipient(more than 5 years at transplant) at transplantation, the presence of recipient CMV-IgG before transplantation, TBI-based conditioning regimen and the presence of acute GvHD(grade ${\geq}II$) were the risk factors for positive CMV antigenemia. In multivariate analysis, unrelated bone marrow transplantation, positive recipient CMV serology and acute GvHD(grade ${\geq}II$) were the independent risk factors for positive CMV antigenemia. Conclusion : Risk factors of CMV infection in children were CMV serostatus of the recipient, the source of stem cells, and acute graft-versus-host disease. The pre-emptive therapy based on CMV antigenemia was effective in the prevention of CMV disease.

• Journal of the Korean Orthopaedic Association
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• v.55 no.3
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• pp.244-252
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• 2020

Purpose: Total femoral replacement (TFR) is an extreme form of limb salvage. Considering the rarity of this procedure, reports have focused on the complications and a proper indication is unclear. This study analyzed 36 patients with TFR who were asked the following: 1) prognostic factors related to survival in patients who underwent TFR with a tumoral cause; 2) overall implant and limb survival; 3) complications, functional outcome, and limb status for patients surviving for more than 3 years. Materials and Methods: According to the causes for TFR, 36 patients were categorized into three groups: extensive primary tumoral involvement (group 1, 15 cases), tumoral contamination by an inadvertent procedure or local recurrence (group 2, 16 cases), and salvage of a failed reconstruction (group 3, 5 cases). The factors that may affect the survival of patients included age, sex, cause of TFR, and tumor volume change after chemotherapy. Results: The overall five-year survival of the 36 patients was 31.5%±16.2%. The five-year survival of 31 patients with tumoral causes was 21.1%±15.6%. The five-year survival of 50.0%±31.0% in patients with a decreased tumor volume after chemotherapy was higher than that of increased tumor volume (p=0.02). The five-year survival of 12 cases with a wide margin was 41.7%±27.9%, whereas that of the marginal margin was 0.0%±0.0% (p=0.03). The ten-year overall implant survival of 36 patients was 85.9%±14.1%. The five-year revision-free survival was 16.6%±18.2%. At the final follow-up, 12 maintained tumor prosthesis, three underwent amputation (rotationplasty, 2; above knee amputation, 1), and the remaining one had knee fusion. Among 16 patients with a follow-up of more than three years, 14 patients underwent surgical intervention and two patients had conservative management. Complications included infection in 10 cases, local recurrences in two cases, and one case each of hip dislocation, bushing fracture, and femoral artery occlusion. Conclusion: Patients showing an increased tumor volume after chemotherapy and having an inadequate surgical margin showed a high chance of early death. In the long-term follow-up, TFR showed a high infection rate and the functional outcome was unsatisfactory. Nevertheless, this procedure is an inevitable option of limb preservation in selected patients.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1193-1200
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• 2005

Purpose : Invasive bacterial infection is a major cause of morbidity and mortality in children. Previously, we reported etiology of invasive infections in healthy children in 1985-1995. This study was performed to update etiology of invasive bacterial infections in the previously healthy children. Methods : We reviewed medical records of 98 episodes of invasive bacterial infections in immunocompetent children at the Seoul National University Children's Hospital in 1996-2004. Results : The frequent pathogens identified over all age groups were Streptococcus pneumoniae (33%) and Staphylococcus aureus(33%). The proportion of Salmonella species and Haemophilus influenzae has been declined to 4% each from 23% and 14%, respectively, compared to previous study. S. agalactiae was the most common isolate in the infants ${\leq}3$ months. Among the infants and children aged 3 months to 2 years and children of 2-5 years, S. pneumoniae(57%, 52%, respectively, in each group) was the most common isolates followed by S. aureus(17% and 24%, respectively). S. aureus was the most common isolates(73%) in children >5 years. Primary bacteremia was the most common clinical diagnosis(27%). S. pneumoniae was responsible for 42% of primary bacteremia, 50% of meningitis, and 69% of bacteremic pneumonia and empyema. S. aureus accounted for 80% of bone and joint infections. The case fatality rate was 8.1% for all invasive infections. Conclusion : We reviewed frequency of bacterial agents of invasive infections in children. The data may be useful for pediatricians to select adequate empirical antibiotics in the management of invasive bacterial infections.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.299-306
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• 2008

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.566-571
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• 2003

Purpose : Leukoencephalopathy(LE) is one of the most serious complications in children with hematologic malignancies during the course of treatment. Early recognition is important to reduce the impact and sequelae from LE. We therefore investigated the clinical features of LE following central nervous system(CNS) prophylaxis in children with hematologic malignancies and evaluated the significance of regular check-ups of brain MRI. Methods : We retrospectively reviewed children with hematologic malignancies who had CNS prophylaxis including intrathecal(IT) methotrexate(MTX) and/or cranial irradiation at the Department of Pediatrics, Kyungpook National University Hospital from Oct. 1995 to May 2002. Fifteen cases of acute leukemia and one case of lymphoma who experienced LE following CNS prophylaxis were included in the study. Clinical data were analyzed from the medical records and brain MRIs were reviewed by neuroradiologists. Results : The ages ranged from 1 to 13 years(median age=5.2 years), and the male to female ratio was 3 : 1. The time interval from the beginning of chemotherapy to the time of diagnosis of LE ranged from 2 to 17 months. They all had IT MTX two to 15 times and ten underwent cranial irradiation(1,800 rads). At the time of diagnosis, ten of them had neuropsychiatric symptoms including seizures, personality changes, headache, etc. After the change of treatment modality, four cases showed significant improvement on follow-up MRIs, six cases had no significant changes and two had worsening of LE. Four patients died of infection and bone marrow relapse. Conclusion : CNS prophylaxis with IT therapy and cranial irradiation may cause leukoencephalopathy during the course of treatment. As a result, regular brain MRI check-up is recommended for the early detection and reducing the incidence of LE, along with changes in the treatment modality.

• Radiation Oncology Journal
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• v.17 no.1
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• pp.43-51
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• 1999

Purpose : To discuss the technical aspect of interstitial brachytherapy including method of implant, insertion time of radioactive source, total radiation dose, and complication, we reviewed patients who had diagnoses of soft tissue sarcoma and were treated by conservative surgery, interstitial implant and external beam radiation therapy Materials and Methods : Between May 1995 and Dec. 1997, ten patients with primary or recurrent soft tissue sarcoma underwent surgical resection (wide margin excision) and received radiotherapy including interstitial brachytherapy. Catheters were placed with regular intervals of 1 ~l.5 cm immediately after tumor removal and covering the critical structures, such as neurovascular bundle or bone, with gelform, muscle, or tissue expander in the cases where the tumors were close to those structures. Brachytherapy consisted of high dose rate, iridium-192 implant which delivered 12~15 Gy to 1 cm distance from the center of source axis with 2~2.5 Gy/fraction, twice a day, starting on 6th day after the surgery, Within one month after the surgery, total dose of 50~55 Gy was delivered to the tumor bed with wide margin by the external beam radiotherapy. Results : All patients completed planned interstitial brachytherapy without acute side effects directly related with catheter implantation such as infection or bleeding. With median follow up duration of 25 months (range 12~41 months), no local recurrences were observed. And there was no severe form of chronic complication (RTOGIEORTC grade 3 or 4). Conclusion : The high dose rate interstitial brachytherapy is easy and safe way to minimize the radiation dose delivered to the adjacent normal tissue and to decrease radiation induced chronic morbidity such as fibrosis by reducing the total dose of external radiotherapy in the management of soft tissue sarcoma with conservative surgery.

• Pediatric Infection and Vaccine
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• v.21 no.2
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• pp.129-138
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• 2014

Purpose: Streptococcus pyogenes is an important cause of invasive diseases in children. We aimed to describe the clinical characteristics of invasive infections due to S. pyogenes in children in Korea. Methods: A retrospective study of children under 18 years of age with invasive infections due to S. pyogenes at Seoul National University Children's Hospital between March 1992 and December 2012, and Seoul National University Bundang Hospital between March 2003 and December 2012 was conducted. Demographic factors, clinical characteristics, laboratory findings, treatment, mortality and morbidity of all patients were reviewed. Results: A total of 30 among 36 cases identified as invasive disease due to S. pyogenes were available for review. There was a predominance for male subjects (male:female=2.75:1). The median age was 50 months (range 12 days to 15 years) and 53.3% were under 5 years of age. Skin and soft tissue infections (9/30, 30.0%), bacteremia without identified focus (4/30, 13.3%) and bone and joint infections (6/30, 20.0%) were the most frequent clinical presentations. Streptococcal toxic shock syndrome (3/30, 10.0%) pulmonary, abdomen and central nervous system infections (2/30, 6.7%) were also seen. There was a peak in number of patients in year 2012 (9/30, 30.0%). There were no cases of mortality. Erythromycin and clindamycin resistance rates were low by 3.8% and 7.5%, respectively. Conclusion: We studied the clinical presentations of invasive infections due to S. pyogenes during the past 20 years in Korean children. The findings of this study help us understand the characteristics of the disease, enhancing early recognition and prompting adequate antibiotic therapy which is important in reducing morbidity and mortality.