Ehlers-Danlos syndrome (EDS) is an inherited disorder of collagen biosynthesis and structure, characterized by skin hyperextensibility, joint hypermobility, aberrant scars, and tissue friability. Besides the skin, skeleton (joint) and vessels, other organs such as the eyes and the intestine can be affected in this syndrome. Accordingly, interdisciplinary cooperation is necessary for a successful treatment. Three basic surgical problems are arising due to an EDS: decreased the strength of the tissue causes making the wound dehiscence, increased bleeding tendency due to the blood vessel fragility, and delayed wound healing period. Surgery patients with an EDS require an experienced surgeon in treating EDS patients; the treatment process requires careful tissue handling and a long postoperative care. A surgeon should also recognize whether the patient shows a resistance to local anesthetics and a high risk of hematoma formation. This report describes a patient with a wide open wound on the foot dorsum and delayed wound healing after the primary approximation of the wound margins.
Anesthetics, Local ; Blood Vessels ; Cicatrix ; Collagen ; Connective Tissue Diseases ; Ehlers-Danlos Syndrome ; Foot ; Hematoma ; Hemorrhage ; Humans ; Intestines ; Joint Instability ; Postoperative Care ; Skeleton ; Skin ; Wound Healing ; Wounds and Injuries

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.
Aged ; Antigens, CD30 ; Diagnosis ; Follow-Up Studies ; Humans ; Lip ; Lymph Nodes ; Lymphoma ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Methotrexate ; Prognosis ; Recurrence ; Skin ; Ulcer

BACKGROUND: When a skin defect occurs, clinicians must work to restore the original skin quality as soon as possible. Accordingly, an artificial dermis can be used to supplement the wound and prevent severe scar contracture formation. The Terudermis is an artificial dermis that is simple and easy to use. We investigated the effectiveness of the Terudermis in the treatment of facial skin defects by analyzing previous relevant cases treated in our institution. METHODS: We retrospectively examined 143 patients who were treated with the Terudermis graft in facial skin defect at Dong Kang General Hospital in 2015 and 2016. The patients’ age, sex and location, wound size, complications were analyzed. In addition, the patients were asked to complete a self-satisfaction questionnaire after 18 months from the completion of treatment. The results were compared with that of autologous full-thickness skin graft (FTSG) and split-thickness skin graft (STSG) patients in same period. RESULTS: The mean self-satisfaction scores evaluated by patients were 4.1±1.0, 4.0±1.3 and 3.5±1.8 for the Terudermis graft, FTSG and STSG patients, respectively. With respect to complications, there were fewer incidences of hematoma, partial skin loss and complete skin loss in the Terudermis graft patients. CONCLUSION: In the present study, the Terudermis, when used to treat post-traumatic facial skin defects, is a good alternative option to obtain satisfactory aesthetic outcomes. Also, the Terudermis grafting is a simple and easy treatment method to perform.
Cicatrix ; Contracture ; Dermis* ; Hematoma ; Hospitals, General ; Humans ; Incidence ; Methods ; Retrospective Studies ; Skin* ; Transplants ; Wounds and Injuries

BACKGROUND: This study aimed to review the long-term clinical outcomes and graft patency of coronary artery bypass grafting (CABG) using arterial grafts in patients with Kawasaki disease (KD) affecting the coronary artery. METHODS: Twenty patients with KD who underwent CABG from January 2002 to June 2014 were enrolled. There were 4 male (20%) and 16 female (80%) patients with ages at operation ranging from 2 to 42 years (median, 17.5 years). Our routine operative strategy was off-pump CABG with arterial grafts. The mean follow-up duration was 59.5 ± 48.5 months (range, 1–159 months). Coronary angiogram or computed tomography angiogram was used to evaluate graft patency in 16 patients (80%). RESULTS: All patients survived CABG without late mortality. Left internal thoracic arteries were used in 19 patients, while right internal thoracic arteries were used in 10 patients. Right gastroepiploic arteries were used in 3 patients, and a saphenous vein graft (SVG) was used in 1 patient. Among the 20 patients, 2 patients underwent coronary reintervention with balloon angioplasty because of graft failure. Two patients underwent coronary reintervention because of new obstructive lesions that were not significant at the time of the initial operation. Patency rates at 5 and 10 years were 94% and 87%, respectively. The rate of freedom from coronary reintervention at 10 years was 82%. CONCLUSION: Off-pump CABG with mainly arterial graft revascularization may be considered a good surgical option for coronary lesions caused by KD.
Angioplasty, Balloon ; Coronary Artery Bypass* ; Coronary Vessels* ; Female ; Follow-Up Studies ; Freedom ; Gastroepiploic Artery ; Humans ; Male ; Mammary Arteries ; Mortality ; Mucocutaneous Lymph Node Syndrome* ; Saphenous Vein ; Transplants

Mitochondrial targeting is a promising approach for solving current issues in clinical application of chemotherapy and diagnosis of several disorders. Here, we discuss direct conjugation of mitochondrial-targeting moieties to anticancer drugs, antioxidants and sensor molecules. Among them, the most widely applied mitochondrial targeting moiety is triphenylphosphonium (TPP), which is a delocalized cationic lipid that readily accumulates and penetrates through the mitochondrial membrane due to the highly negative mitochondrial membrane potential. Other moieties, including short peptides, dequalinium, guanidine, rhodamine, and F16, are also known to be promising mitochondrial targeting agents. Direct conjugation of mitochondrial targeting moieties to anticancer drugs, antioxidants and sensors results in increased cytotoxicity, anti-oxidizing activity and sensing activity, respectively, compared with their non-targeting counterparts, especially in drug-resistant cells. Although many mitochondria-targeted anticancer drug conjugates have been investigated and , further clinical studies are still needed. On the other hand, several mitochondria-targeting antioxidants have been analyzed in clinical phases I, II and III trials, and one conjugate has been approved for treating eye disease in Russia. There are numerous ongoing studies of mitochondria-targeted sensors.

BACKGROUND: Nasal bone fracture is one of the most common facial bone fracture types, and the surgical results exert a strong influence on the facial contour and patient satisfaction. Preventing secondary deformity and restoring the original bone state are the major goals of surgeons managing nasal bone fracture patients. In this study, a treatment algorithm was established by applying the modified open reduction technique and postoperative care for several years. METHODS: This article is a retrospective chart review of 417 patients who had been received surgical treatment from 2014 to 2015. Using prepared questionnaires and visual analogue scale, several components (postoperative nasal contour; degree of pain; minor complications like dry mouth, sleep disturbance, swallowing difficulty, conversation difficulty, and headache; and degree of patient satisfaction) were evaluated. RESULTS: The average scores for the postoperative nasal contour given by three experts, and the degree of patient satisfaction, were within the “satisfied” (4) to “very satisfied” (5) range (4.5, 4.6, 4.5, and 4.2, respectively). The postoperative degree of pain was sufficiently low that the patients needed only the minimum dose of painkiller. The scores for the minor complications (dry mouth, sleep disturbance, swallowing difficulty, conversation difficulty, headache) were relatively low (36.4, 40.8, 65.2, 32.3, and 34 out of the maximum score of 100, respectively). CONCLUSION: Satisfactory results were obtained through the algorithm-oriented management of nasal bone fracture. The degree of postoperative pain and minor complications were considerably low, and the degree of satisfaction with the nasal contour was high.
Classification* ; Congenital Abnormalities ; Deglutition ; Facial Bones ; Headache ; Humans ; Mouth ; Nasal Bone* ; Pain, Postoperative ; Patient Satisfaction ; Postoperative Care ; Retrospective Studies ; Surgeons

This study compared the efficacy of DA-9601 (Dong-A ST Co., Seoul, Korea) and its new formulation, DA-5204 (Dong-A ST Co.), for treating erosive gastritis. This phase III, randomized, multicenter, double-blind, non-inferiority trial randomly assigned 434 patients with endoscopically proven gastric mucosal erosions into two groups: DA-9601 3 times daily or DA-5,204 twice daily for 2 weeks. The final analysis included 421 patients (DA-5204, 209; DA-9601, 212). The primary endpoint (rate of effective gastric erosion healing) and secondary endpoints (cure rate of endoscopic erosion and gastrointestinal [GI] symptom relief) were assessed using endoscopy after the treatment. Drug-related adverse events (AEs), including GI symptoms, were also compared. At week 2, gastric healing rates with DA-5204 and DA-9601 were 42.1% (88/209) and 42.5% (90/212), respectively. The difference between the groups was −0.4% (95% confidence interval, −9.8% to 9.1%), which was above the non-inferiority margin of −14%. The cure rate of gastric erosion in both groups was 37.3%. The improvement rates of GI symptoms with DA-5204 and DA-9601 were 40.4% and 40.8%, respectively. There were no statistically significant differences between the two groups in both secondary endpoints. AEs were reported in 18 (8.4%) patients in the DA-5204 group and 19 (8.8%) in the DA-9601 group. Rates of AE were not different between the two groups. No serious AE or adverse drug reaction (ADR) occurred. These results demonstrate the non-inferiority of DA-5204 compared to DA-9601. DA-5204 is as effective as DA-9601 in the treatment of erosive gastritis. Registered randomized clinical trial at ClinicalTrials.gov (NCT02282670)
Artemisia ; Double-Blind Method ; Drug-Related Side Effects and Adverse Reactions ; Endoscopy ; Gastritis* ; Humans ; Seoul

This article provides an overview of the developmental history and rationale of medical ethics to establish the code of ethics and professional conduct of the Korean Academy of Child and Adolescent Psychiatry (KACAP). Most medical professional organizations have their own codes of ethics and conduct because they have continuous responsibility to regulate professional activities and conducts for their members. The Ethics and Award Committee of the KACAP appointed a Task-Force to establish the code of ethics and conduct in 2012. Because bioethics has become global, the Ethics Task Force examined global standards. Global standards in medical ethics and professional conduct adopted by the World Medical Association and the World Psychiatric Association have provided the basic framework for our KACAP's code of ethics and professional conduct. The Code of Ethics of the Americal Academy of Child and Adolescent Psychiatry has provided us additional specific clarifications required for child and adolescent patients. The code of ethics and professional conduct of the KACAP will be helpful to us in ethical clinical practice and will ensure our competence in recognizing ethical violations.
Adolescent ; Adolescent Psychiatry* ; Adolescent* ; Advisory Committees ; Awards and Prizes ; Bioethics ; Child* ; Codes of Ethics ; Ethics ; Ethics, Medical* ; Humans ; Mental Competency ; Societies

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and fludarabine reduced-toxicity myeloablative conditioning. Intravenous busulfan was administered once daily for 4 consecutive days (days –8 to –5), and the target area under the curve was 75,000 µg·hr/L. Fludarabine (40 mg/m2) was administered once daily for 6 consecutive days from days –8 to –3. Antithymocyte globulin (2.5 mg/kg/day) was administered from days –4 to –2. The patient underwent successful engraftment and did not have any severe toxicity related to the transplantation. Conditioning with a targeted busulfan and fludarabine regimen could provide a better outcome for HSCT in CGD, with close regulation of the busulfan dose.
Antilymphocyte Serum ; Bone Marrow Transplantation* ; Bone Marrow* ; Busulfan* ; Granulomatous Disease, Chronic* ; Hematopoietic Stem Cell Transplantation ; Humans ; Mortality ; Transplantation Conditioning

Hypothyroid myopathy is observed frequently and the resolution of the clinical manifestations of myopathy following thyroid hormone replacement is well known. However, a specific subtype of hypothyroid myopathy, Hoffmann's syndrome, characterized by increased muscular mass (pseudohypertrophy), proximal muscle weakness, muscle stiffness and cramps, is rarely reported. Herein, we describe a 34-year-old male who presented with proximal muscle weakness and non-pitting edema of the lower extremities. He initially visited the neurology department where he was suspected of having polymyositis. Additional laboratory evaluation revealed profound autoimmune hypothyroidism and elevated muscle enzymes including creatine kinase. The patient was started on levothyroxine treatment and, subsequently, clinical symptoms and biochemical parameters resolved with the treatment. The present case highlights that hypothyroidism should be considered in the differential diagnosis of musculoskeletal symptoms even in the absence of overt manifestations of hypothyroidism. To our knowledge, this is the first case reported in Korea.
Adult ; Creatine Kinase ; Diagnosis, Differential ; Edema ; Humans ; Hypothyroidism ; Korea ; Lower Extremity ; Male ; Muscle Cramp ; Muscle Weakness ; Muscular Diseases* ; Neurology ; Polymyositis ; Thyroid Gland ; Thyroxine