• The Journal of the Korean bone and joint tumor society
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• v.2 no.1
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• pp.8-17
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• 1996

The purpose of this study is a comparative evaluation of range motion, especially extension deficit between the group of total patellectomy and that of intact patella, after reconstruction of the patellar tendon in the prosthetic replacement of a proximal tibia. Between 1990 and 1994, 15 patients who had a primary malignancy on proximal tibia were operated on. All patients were evaluated clinically and radiographically. Two patients were excluded because one had a deep infection treated with arthrodesis of the knee and the other was a composite allograft. The mean follow-up of the 13 patients was 27 months(15-47), including 10 osteosarcomas, 1 chondrosarcoma, 1 malignant fibrous histiocytoma and 1 malignant giant cell tumor. Eleven patients had a resection of the proximal tibia and 2 had an extracapsular total knee resection with distal femur. Reconstruction of the defect was done in 8 cases with a custom-made Link Endo-Model Total Rotation Knee Joint Prosthesis, and in 5 with How Medica Modular Resection System (HMRS). We used two methods to reconstruct the ligamentum patellae. Fixation of the patellar tendon to the prosthesis only with suturing and/or stapling(group SS) was done in 7. Transposition of gastrocnemius muscle to enhance fixation and to cover the prosthesis(group TG) was done in 6. Regardless of fixation methods, total patellectomy was done in 5 either to lengthen the patellar tendon or to make primary skin closure easier or for both. In 8 cases, patella was left intact or resurfaced with polyethylene prosthesis. Active extension was measured while the patient was in a sitting position. There is no statistically meaningful difference in terms of extension deficit (Wilcoxon rank test, p=0.8800) between patellectomy group and intact patella group, and between group of fixation only with suturing and that of gastrocnemius transposition. Two cases of extension deficit over 30 degree were seen in group SS and in the group of intact patella. Conclusively, total patellectomy could be an option without increasing the risk of extension deficit when primary skin closure is difficult or patellar tendon is a little bit short to be fixed. There is no rating in the Enneking system of functional evaluation that this finding into consideration.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.32 no.5
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• pp.410-417
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• 2006

The present study was aimed to compare the resorption rate and the histological change of the autogenous dermis and the artificial dermis (Terudermis$^{(R)}$) after the transplantation, and to report the clinical results of the use of Terudermis$^{(R)}$ in order to restore the soft tissue defect. Twenty mature rabbits, weighing about 2 kg, were used for the experimental study. The autogenous dermis and the Terudermis$^{(R)}$ size 1${\times}$1 cm were transplanted to the space between the external abdominal oblique muscle and the external abdominal oblique fascia of the each rabbits. They were divided into 4 groups (n=5 each) and gathered at 1, 2, 4, and 8 weeks after the transplantation. The resorption rate was calculated, and H-E stain was preformed to observe the histological changes. The chart review of the 17 patients who received Terudermis$^{(R)}$ graft to the facial soft tissue defects was conducted for the clinical study. The resorption rate at 8 weeks after the transplantation was 21.5% for the autogenous dermis, and 36.4% Terudermis$^{(R)}$. In microscopic examinations, the infiltration of the inflammatory cells and the epidermal inclusion cyst were observed in the autogenous dermis graft. The neovascularization and the progressive growth of the new fibroblast were shown in the Terudermis$^{(R)}$ graft. In clinical data of 17 patients, the size of the grafted Terudermis$^{(R)}$ was from 1.5$cm^2$ to 7.5$cm^2$ (average 3.5$cm^2$). Follow-up ranged from 5 to 25 months. Fourteen patients with cleft palate demonstrated stability of the graft and unremarkable complications. But unstability of the graft and the partial relapse were observed in three patients received the vestibuloplasty. These results indicate that Terudermis$^{(R)}$ can be available substitute of autogenous dermis because of the stability about resorption, the histocompatibility, and the unremarkable clinical complications.

• Archives of Plastic Surgery
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• v.38 no.1
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• pp.27-34
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• 2011

Purpose: Anterolateral thigh (ALT) perforator free flap is commonly used because of its various benefits. The author reports important factors of preoperative and perioperative ALT perforator free flap and causes of failure. Methods: 84 patients who were treated with ALT perforator free flap from December 2004 to 2008, and February 2010 to April 2010 were studied. 61 patients were male and 23 were female. The mean age of patients was 51.1. The main cause was neoplasm and the main reconstructive areas were head and neck area. The size of flap was various from $3{\times}4$ to $12{\times}18$ cm. 6 patients received split thickness skin graft at donor site. Preoperative angiography was checked to all patients. Results: Among the 84 patients, partial necrosis of flaps occurred in 4 patients because of atherosclerosis, varicose vein, or inattention of patient, etc. And total flap necrosis in 5 patients because of abnormal vessels of recipient area or delay of operation, etc. One case of serous cyst was found as the complication of donor area. Two cases of skin graft on donor site were done because of suspected muscle compartment syndrome, 4 cases of that because of large flap. Septocutaneous perforators were found in 7 cases. The author couldn't find reliable perforator in 3 cases, ipsilateral anteromedial thigh perforator and contralateral ALT perforator and latissimus dorsi musculocutaneous free flap were done instead of ALT. There was no case which needed reoperation because of the impairment of blood supply, and 3 cases were revised by leech because of the burn injury by a lamp or venous congestion. Conclusion: Although ALT perforator free flap is widely used with its various merits, many factors such as preoperative condition of donor or recipient area, morphology of defect and operating time need to consider to prevent flap necrosis. And operators should need careful technique because septocutaneous perforator is uncommon, and musculocutaneous perforator is common but difficult to dissect.

• Neonatal Medicine
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• v.17 no.2
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• pp.254-261
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• 2010

Little is known about neonatal mitochondrial disease, though mitochondrial metabolic disorders may often present in the neonatal period because of the high energy requirement of neonate. In newborn period, common presentations are not specific and the disease course may be rapid and fatal. In this study, we report three cases of neonatal mitochondrial disease. The first case was strongly suspected because of sudden seizure and mental change with severe lactic acidosis, and multiorgan failure. Plasma lactate/pyruvate (L/P) ratio was increased to 55.6 with marked lactic aciduria and increased plasma alanin up to 2,237 nmol/mL. In the second patient, a peritoneal dialysis was performed for acute adrenal and renal failure, but metabolic acidosis persisted. Plasma L/P ratio was increased to 23.9, and MRC I (mitochondrial respiratory chain defect) was diagnosed through the enzymatic analysis of the muscles. The third case showed repetitive episode of lactic acidosis during the first two months of life, hypotonia, failure to thrive and feeding difficulties. We found markedly increased cerebrospinal fluid L/P ratio up to 57 though plasma L/P ratio(19.4) was borderline with increased plasma lactate. The lactate peak was prominent in brain magnetic resonance spectroscopy (MRS). MRC II was confirmed through muscle biopsy. Plasma lactate level and lactate peak of brain MRS were normalized after conservative treatment.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.213-217
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• 2007

Pompe disease is a genetic disorder caused by a deficiency of acid ${\alpha}$-glucosidase (GAA). Infantile onset Pompe disease is uniformly lethal. Affected infants generally present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, which is rapidly followed by death, usually by the age of one. The late-onset form is characterized less severe symptoms and prognosis. Therapy for Pompe disease is intended to directly address the underlying metabolic defect via intravenous infusions of recombinant human GAA to replace the missing enzyme. We report a case of atypical infantile-onset Pompe disease that presented symptoms in infancy but had less severe clinical manifestations and improved after GAA enzyme replacement ($Myozyme^{(R)}$, Genzyme Co., MA, USA) therapy. It is very important that pediatricians become aware of signs and symptoms of Pompe disease, such as a nasal voice or a waddling gait at an early stage so that these patients can benefit from appropriate GAA replacement therapy as soon as possible.

• The Korean Journal of Physiology and Pharmacology
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• v.5 no.5
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• pp.397-405
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• 2001

The ryanodine receptor, a $Ca^{2+}$ release channel of the sarcoplasmic reticulum (SR), is responsible for the rapid release of $Ca^{2+}$ that activates cardiac muscle contraction. In the excitation-contraction coupling cascade, activation of SR $Ca^{2+}$ release channel is initiated by the activity of sarcolemmal $Ca^{2+}$ channels, the dihydropyridine receptors. Previous study showed that the relaxation defect of diabetic heart was due to the changes of the expressional levels of SR $Ca^{2+}$ATPase and phospholamban. In the diabetic heart contractile abnormalities were also observed, and one of the mechanisms for these changes could include alterations in the expression and/or activity levels of various $Ca^{2+}$ regulatory proteins involving cardiac contraction. In the present study, underlying mechanisms for the functional derangement of the diabetic cardiomyopathy were investigated with respect to ryanodine receptor, and dihydropyridine receptor at the transcriptional and translational levels. Quantitative changes of ryanodine receptors and the dihydropyridine receptors, and the functional consequences of those changes in diabetic heart were investigated. The levels of protein and mRNA of the ryanodine receptor in diabetic rats were comparable to these of the control. However, the binding capacity of ryanodine was significantly decreased in diabetic rat hearts. Furthermore, the reduction in the binding capacity of ryanodine receptor was completely restored by insulin. This result suggests that there were no transcriptional and translational changes but functional changes, such as conformational changes of the $Ca^{2+}$ release channel, which might be regulated by insulin. The protein level of the dihydropyridine receptor and the binding capacity of nitrendipine in the sarcolemmal membranes of diabetic rats were not different as compared to these of the control. In conclusion, in diabetic hearts, $Ca^{2+}$ release processes are impaired, which are likely to lead to functional derangement of contraction of heart. This dysregulation of intracellular $Ca^{2+}$ concentration could explain for clinical findings of diabetic cardiomyopathy and provide the scientific basis for more effective treatments of diabetic patients. In view of these results, insulin may be involved in the control of intracellular $Ca^{2+}$ in the cardiomyocyte via unknown mechanism, which needs further study.

• Archives of Plastic Surgery
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• v.38 no.2
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• pp.199-202
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• 2011

Purpose: Hyoid bone is a U-shaped bone in the anterior of the neck. Hyoid bone fractures are exceedingly rare and represent only 0.002% of all fractures because of its protective position relative to the mandible and its suspension by elastic musculature. We report a patient who presented hyoid bone fracture associated with hypoglossal nerve palsy. We also discuss the possible complication and treatment. Methods: A 69-year-old man was transferred from another institution because of persistent purulent discharge from the left chin. He had a history of trauma in which a knuckle crane grabbed his face and neck in the construction site. A CT scan at the time of the accident demonstrated a comminuted fracture of the right side of the mandible and hyoid bone fracture at the junction between body and right greater cornua. The displaced fracture of hyoid bone and fullness in the pre-epiglottic space were noted, probably indicating some edema. The patient was transferred into ICU after treatment of emergency tracheostomy because the patient showed respiratory distress rapidly. When the patient was hospitalized in our emergency room, he complained of dysphagia and pain when swallowing. On examination of oral cavity, the presence of muscle wasting with fasciculation of the tongue was noted and the tongue deviates to the left side on protruding from the mouth. Pharyngolarygoscopy was performed to make sure that there was no evidence of progressive swelling and pharyngeal laceration. Results: The patient underwent surgical removal of dead and infected tissue from the wound and reconstruction of mandibular bony defect by iliac bone grafting. Hyoid bone fracture was managed conservatively with oral analgesics, soft diet and restricted movement. Hypoglossal nerve palsy was resolved within 7 weeks after trauma without complications. Conclusion: Closed hyoid bone fracture is usually uncomplicated and thus it can be treated conservatively. Surgical intervention for hyoid bone fracture is recommended for patient with airway compromise, pharyngeal perforation and painful symptoms which show no response to conservative care. Furthermore, since respiratory distress syndrome may develop quickly, close observation is required. Besides, hypoglossal nerve palsy is a rarely recognized complication of hyoid bone fracture.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.33 no.2
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• pp.114-120
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• 2007

The augmentation of soft tissue defects is one of the critical problems in the oral and maxillofacial surgery. Various types of graft materials, both autologous and non-autologous, have been used for the augmentation of soft tissue in the facial region. However, it is not easy to choose an ideal material for soft tissue augmentation because each has its advantages and disadvantages. An ideal graft material should meet the following criteria : it should not leave a scar at the area from which it was taken; should have less likelihood of causing infection; should feel natural after implanted; and should be not absorbed. Among the materials meeting these criteria, human dermis and artificial dermis are commonly used for clinical purposes. The present study was aimed to investigate and compare the resorption rate and the histological change following the use of the autologous dermis, the human homogenous dermis $Alloderm^{(R)}$, and the artificial dermis $Terudermis^{(R)}$ to reconstruct the soft tissue defect. Twenty mature rabbits of either sex, weighing about 2 ㎏, were used. Each rabbit was transplanted with the autologous dermis, $Alloderm^{(R)}$, and $Terudermis^{(R)}$ size $1{\times}1-cm$ at the space between the external abdominal oblique muscle and the external abdominal oblique fascia. They were then divided into 4 groups (n=5 each) according to the time elapsed after the surgery: 1, 2, 4, and 8 weeks. The resorption rate was calculated by measuring the volume change before and after the transplantation, and H-E stain was preformed to observe the histological changes. The resorption rate after 8 weeks was 21.5% for the autologous dermis, 16.0% $Alloderm^{(R)}$, and 36.4% $Terudermis^{(R)}$, suggesting that $Alloderm^{(R)}$ is the most stable while $Terudermis^{(R)}$ is the most unstable. In microscopic examinations, the autologous dermis graft was surrounded by inflammatory cells and showed foreign body reactions. The epidermal inclusion cyst was observed in the autologous dermis graft. $Terudermis^{(R)}$ and $Alloderm^{(R)}$ demonstrated neovascularization and the progressive growth of new fibroblast. The results suggest that $Terudermis^{(R)}$ and $Alloderm^{(R)}$ can be availably for substituting the autologous dermis.

• Journal of Periodontal and Implant Science
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• v.35 no.2
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• pp.335-343
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• 2005

Inorganic bovine bone mineral has been widely researched as bone substitution materials in orthopedic and oral and maxillofacial application. OCS-B(NIBEC, Korea) is newly-developed inorganic bovine bone mineral. The aim of this study is to evaluate the safety and efficacy of bovine bone-derived bone graft material(OCS-B). Micro-structure of newly-developed inorganic bovine bone mineral(OCS-B) was analyzed by scanning electron microscope(SEM). Round cranial defects with eight mm diameter were made and filled with OCS-B in rabbits. OCS-B was inserted into femoral quadrant muscle in mouse. In scanning electron microscope, OCS-B was equal to natural hydroxyapatite. Rabbits were sacrificed at 2 weeks and 4 weeks after surgery and mice were sacrificed at 1 week and 2 weeks after surgery. Decalcified specimens were prepared and observed by microscope. In calvarial defects, osteoid and new bone were formed in the neighborhood of OCS-B at 2 weeks after surgery. And at 4 weeks after surgery osteoid and new bone bridge formed flourishingly. No inflammatory cells were seen on the surface of OCS-B at 1 week and 2 weeks in mouse experimental group. It is concluded that newly-developed inorganic bovine bone mineral(OCS-B) is a flourishing bone-forming material and biocompatible material.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.292-298
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• 2009

Background: Surgery for mitral valve disease in children carries both technical and clinical difficulties that are due to both the wide spectrum of morphologic abnormalities and the high incidence of associated cardiac anomalies. The purpose of this study is to assess the outcome of mitral valve surgery for treating congenital mitral regurgitation in children. Material and Method: From 1997 to 2007, 22 children (mean age: 5.4 years) who had congenital mitral regurgitation underwent mitral valve repair. The median age of the patients was 5.4 years old and four patients (18%) were under 12 months of age. 15 patients (68%) had cardiac anomalies. There were 13 cases of ventricular septal defect, 1 case of atrial septal defect and 1 case of supravalvar aortic stenosis. The grade of the preoperative mitral valve regurgitation was II in 4 patients, III in 15 patients and IV in 3. The regurgitation was due to leaflet prolapse in 12 patients, annular dilatation in 4 patients and restrictive leaflet motion in 5 patients. The preoperative MV Z-value and the regurgitation grade were compared with those obtained at follow-up. Result: MV repair was possible in all the patients. 19 patients required reduction annuloplasty and 18 patients required valvuloplasty that included shortening of the chordae, papillary muscle splitting, artificial chordae insertion and cleft closure. There were no early or late deaths. The mitral valve regurgitation after surgery was improved in all patients (absent=10, grade I=5, II=5, III=2). MV repair resulted in reduction of the mitral valve Z-value ($2.2{\pm}2.1$ vs. $0.7{\pm}2.3$, respectively, p<0.01). During the mid-term follow-up period of 3.68 years, reoperation was done in three patients (one with repair and two with replacement) and three patients showed mild progression of their mitral reguration. Conclusion: our experience indicates that mitral valve repair in children with congenital mitral valve regurgitation is an effective and reliable surgical method with a low reoperation rate. A good postoperative outcome can be obtained by preoperatively recognizing the intrinsic mitral valve pathophysiology detected on echocardiography and with the well-designed, aggressive application of the various reconstruction techniques.