• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.303-329
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• 2019

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.

• The Journal of the Korean bone and joint tumor society
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• v.12 no.1
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• pp.37-46
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• 2006

Purpose: To evaluate the end results between the surgical treatment with neo-adjuvant chemotherapy in Korea and non-invasive high intensity focused ultrasound (HIFU) technique in China for osteogenic sarcomas. Materials and Methods: The surgical treatment with neoadjuvant chemotherapy for total 67 cases (4 IIA, 58 IIB, and 5 III) in Korea since 1993 and the HIFU therapy for total 71 cases (57 II and 14 III) in China since 1997 was performed. In Korea, neo-adjuvant chemotherapy in 66 cases out of total 67 patients, but the adjuvant chemotherapy in only one case was done. On the contrary, in China, full chemotherapy for more than 9 times for 37 patients with stage II out of total 71 cases, but for less than 8 times of partial chemotherapy for 23 patients (stage II) and 14 patients (stage III) was done. The surgical treatment in total 67 Korean patients was done with wide resection and reconstruction for 56 patients, but wide resection without reconstruction for 5 patients and amputation for 6 patients. In china, total 71 patients was treated with average 1.5 times (1~4 times) of HIFU, and if there are some evidences of residual tumor after HIFU with following MRI, the second HIFU therapy was given 2~4 weeks later. After then, the bony defect was in no touch, keep bracing for long time expecting regeneration. All of them were followed for average 46 Mo (12~150 Mo) in Korea, but followed for average 22 Mo (9 years~8 months) in China. Results: The 5 year survival rate (stage II), was average 92.7% (IIA 100%, IIB 85.5%) in Korea series, and average 78.7% (full chemotherapy 91.8%, part chemotherapy 56.6%) in China series. The 3 year survival rate (stage III) was 20% in Korea and 7.1% in China. So, the final overall survival rate was 65.2% in Korea and 51.8% in China. The overall functional outcome score by ISOLS was 24.3 (81%) in Korea and 19.8 (73%) in China. There are 25.4% (17/67 cases) of complications in Korea and 31% (27 complications in 22 patients out of total 71 cases) in China after each treatment. Conclusions: The end results of Korea series which was treated with neoadjuvant chemo- and surgical methods are better than that of non-invasive thermal ablation in china. But we also believe the HIFU, as one of, was also effective to decrease the local recurrence and symptomatic releaf for stage II or even in III of osteogenic sarcomas.

• 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.