• Journal of Dental Anesthesia and Pain Medicine
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• v.22 no.6
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• pp.451-455
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• 2022

Glycogen storage disease (GSD) is a group of inherited disorders, which result in the deficiency of enzymes involved in glycogen metabolism, leading to an accumulation of glycogen in various organs. Deficiency of amylo-1-6-glicosidase (debranching enzyme) causes glycogen storage disease type III (GSD III). The main problems that anesthesiologists face in patients with GSD III include hypoglycemia, muscle weakness, delayed awakening due to abnormal liver function, possible difficulty in airway, and cardiomyopathy. In the face of these difficulties, airway preparation and appropriate glucose monitoring and support during the fasting period are important. The doses of the drugs to be used should be calculated considering the increased volume of distribution and decreased metabolic activity of the liver. We present the case of a child with GSD IIIa who underwent dental prosedation under general anesthesia. She was also being prepared for liver transplantation. This case was additionally complicated by the patient's serious allergic reaction to eggs and milk.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.192-198
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• 2002

Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced a case of Alagille syndrome in a 10 month-old male presenting with jaundice. He had chronic cholestasis, characteristic face, cardiovascular abnormalities (aortic stenosis, dextrocardia, double chamber of left ventricle), and situs inversus. Histological examination of liver biopsy specimen revealed paucity of interlobular bile ducts with septal fibrosis, cirrhotic transformation and severe cholestasis. He underwent liver transplantation, but died of cardiopulmonary arrest associated with cardiac anomaly.

• Archives of Plastic Surgery
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• v.38 no.3
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• pp.245-250
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• 2011

Purpose: Composite tissue allotransplantation has emerged as a new therapeutic modality to reconstruct major tissue defects of the head, neck and extremities. A questionnaire-based instrument, the Louisville Instrument for Transplantation (LIFT), has been developed to objectively assess the risk-versus-benefit ratio for composite tissue allotransplantation procedures. The objective of this study is to assess if the LIFT is a useful, reliable and valid tool to apply to the Korean population. Methods: Seventy-three medical students and 60 lay public completed the LIFT questionnaire (translated to Korean) over the period from February 2010 to April 2010. Internal consistency was assessed using Cronbach's alpha. Test-retest reliability was analyzed using Pearson's correlation coefficient. Construct validity was assessed by comparing Pearson's correlation coefficients between perceived improvements in quality of life and responses to risk tolerance questions concerning organ transplants. Results: Measurements of the test-retest reliability showed that Pearson's correlation coefficients ranged from 0.241 to 0.902, and Cronbach's alphas ranged from 0.52 to 0.80 for medical students and from 0.63 to 0.83 for the lay public. Pearson's correlation coefficients showed significant correlations between perceived improvements in quality of life and responses to risk tolerance questions concerning organ transplants. Hand transplant showed a significant correlation in medical students. Foot, hand, two hands, larynx, partial face transplants showed significant correlations for the lay public. Conclusion: The applicability of the LIFT to the Korean population was found to be reliable and valid. The LIFT may serve as a useful tool for clinical application in the Korean population.

• Annals of dermatology
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• v.30 no.6
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• pp.704-707
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• 2018

Parry Romberg Syndrome (PRS), also known as idiopathic progressive hemifacial atrophy, is a rare neurocutaneous disorder characterized by loss of skin and subcutaneous fat of face, muscles, and bones causing unilateral atrophy. Most patients require only soft tissue augmentation although syndrome has varying grades of severity. In the majority of reported cases, it has been treated with surgical flap or autologous fat transplantation. However, these treatments need complicated surgical skills which take a lot of time and cost. Herein we report the first case of PRS augmented by hyaluronic acid (HA) filler in a 42-year-old female patient to suggest that HA filler could be a safe, simple, and even rational economic alternative to surgical treatment.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.35 no.5
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• pp.342-351
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• 2013

Advances in immunosuppressive treatments and microsurgical techniques have rendered composite tissues allotransplantation (CTA), such as heteregeneous or non-organ tissues, possible in humans. CTA has evolved dramatically since the first successful rat hind limb allotransplantation. Numerous clinical applications including face, hand, trachea, larynx, and vascularized joint have been performed. Although composite tissue allografts are still in their infancy, they have opened a new era in the field of transplantation surgery and pathology, so that maxillofacial reconstructive surgeons may occasionally be faced with the challenge of diagnosing skin refection of a composite tissue allograft. Facial allotransplantation (FAT) is a new surgical technique that could be considered as a new paradigm in facial reconstruction. Since the first human FAT had been achieved in 2005, 17 cases have been reported in the world up to date. However, many problems such as life-long immunosuppression, immune rejection, ethical problems and psychological problems are remained, so facial CTA is new reconstructive option with no general acceptance. The authors reviewed the indications, the results of 17 cases and their complications, and additional consideration factors in this article, and intended to raise the awareness of oral and maxillofacial surgeons in this type of facial transplantation.

• Archives of Craniofacial Surgery
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• v.12 no.2
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• pp.86-92
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• 2011

Purpose: The purpose of this study was to investigate the possibility that a dynamic facial composite flap with sensory and motor nerves could be made available from donor facial composite tissue. Methods: The faces of 3 human cadavers were dissected. The authors studied the donor faces to assess which facial composite model would be most practicable. A "panorama facial flap" was excised from each facial skeleton with circumferential incision of the oral mucosa, lower conjunctiva and endonasal mucosa. In addition, the authors measured the available length of the arterial and venous pedicles, and the sensory nerves. In the recipient, the authors evaluated the time required to anastomose the vessels and nerve coaptations, anchor stitches for donor flaps, and skin stitches for closure. Results: In the panorama facial flap, the available anastomosing vessels were the facial artery and vein. The sensory nerves that required anastomoses were the infraorbital nerve and inferior alveolar nerve. The motor nerve requiring anstomoses was the facial nerve. The vascular pedicle of the panorama facial flap is the facial artery and vein. The longest length was 78 mm and 48 mm respectively. Sensation of the donor facial composite is supplied by the infraorbital nerve and inferior alveolar nerve. Motion of the facial composite is supplied by the facial nerve. Some branches of the facial nerve can be anastomosed, if necessary. Conclusion: The most practical facial composite flap would be a mid and lower face flap, and we proposed a panorama facial flap that is designed to incorporate the mid and lower facial skin with and the unique tissue of the lip. The panorama facial composite flap could be considered as one of the practicable basic models for facial allotransplantation.

• Archives of Plastic Surgery
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• v.38 no.3
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• pp.295-299
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• 2011

Purpose: It is generally believed that alopecia is caused by various factors such as scars, stress, genetical factors, androgens, etc. Androgenic alopecia is one of the most common cause of alopecia and found mainly in males. Propecia (Merck & Co., USA) and Minoxidil (McNEIL-PPC, Inc, USA) were the drugs approved from FDA for treatment of androgenic alopecia. Surgical treatments such as flap, tissue expansion, scalp reduction and hair transplantation can be considered if necessary. Hair micrograft techniques were developed for natural hair shapes and minimal adverse effect. There were attempts to measure the length of the forehead of the Korean young adults. However attempts to classify the shape and location of forehead hairline were rare. This study attempted to find out standard hairlines of young adults in their 20s & 30s and the result would be the guideline of the hairline in hair replacement surgery of male patients in their 40s & 50s. Methods: 200 male adults in 20s and 30s were photographed and measured the length of 11 vertical index lines to determine hairline. The indexes are the distances from hairline to intercanthal midpoint (A), to medial canthus (B), to upper eyelid fissure (C), to lower eyelid fissure (D), to lateral canthus (E) and distance from lateral highest point to medial lowest point, if the hairline is M-shape (F). Additionally, we classified the hairlines into 4 groups, M, horizontal, inverted U and irregular shapes. Results: The most common hairline of male adults in their 20s is inverted U-shape (53.3%), followed by horizontal-shape, M-shape, irregular-shape. In their 30s, inverted U-shape (59%) is followed by irregular-shape, M-shape, horizontal-shape. The M-shape is more frequently found in males in 30s than those in 20s. The mean values of the indexes in their 20s are as follows: A (76.14 mm), B (Rt: 75.78 mm, Lt:76.41 mm), C (Rt: 69.43 mm, Lt: 69.92 mm), D (Rt: 76.92 mm, Lt:77.46 mm), E (Rt: 64.16 mm, Lt: 64.73 mm), F (4.09 mm). Those in their 30s are as follows: A (76.13 mm), B (Rt: 76.114 mm, Lt: 76.02 mm), C (Rt: 69.87 mm, Lt: 70.37 mm), D (Rt: 77.37 mm, Lt: 77.58 mm), E (Rt: 69.63 mm, Lt: 69.85 mm), F (6.14 mm). Conclusion: The knowledge about human body measurement is indispensable to plastic surgeons. In this study, inverted U shape is the most common type of hairline in 30s, and similar distribution is found in 20s. The percentage of M shape in their 30s is elevated more than 10% compared to that in their 20s. The study of hairline shapes and 11 indexes of hairlines can be useful for planning of the hair transplantation and postoperative evaluation. This study being based on photogrammetry, there may be differences between actual distance of curved face and projected distance on flat photographs.

• Korean Journal of Head & Neck Oncology
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• v.36 no.1
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• pp.39-44
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• 2020

Xeroderma pigmentosum is a rare autosomal recessive disease, related to defects in DNA repair mechanism. It presents skin lesions on sun-exposed areas, leading to various skin cancer. Skin lesions can be treated with cryotherapy, skin resurfacing, 5-FU, Imiquimod, topical T4 endonuclease V, radiotherapy and genetic therapy, but invasive skin cancer should be treated by a surgery. We report a 12-year-old female xeroderma pigmentosum patient with recurrent basal cell carcinoma successfully treated by skin grafting. In that there is no cure for this disease, prevention and patient education is most important.

• Journal of Korean Academy of Nursing
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• v.46 no.2
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• pp.271-282
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• 2016

Purpose: This study was done to explore adaptation experience of living kidney donors after donation. Specific aims were to identify challenges donors face in the process of adaptation following surgery and how they interact with recipients and other people. Methods: Grounded theory methodology was utilized. Participants were 13 living kidney donors at six months or more after donation. Data were collected by in-depth interviews with individual participants. Data were analyzed using constants comparative method with theoretical saturation. Results: A core category emerged as 'keeping the fences of my family in spite of vulnerability'. The adaptation process after donation was manifested in four phases: exploration, balance, maintenance, and acclimatization. Phenomenon was perception of vulnerability. Strategies to manage the vulnerability were assessing changes of body awareness, tailoring regimen to one's own body condition, coping with health problems, keeping restoration of health, and ruminating on the meaning of one's kidney donation. Consequences were reestablishing family well-being, realizing the values of one's kidney donation, and living with uncertainty. Conclusion: Findings of the study indicate that there is a need for health professionals to understand the vulnerability of living kidney donors and help their family system maintain a healthy and productive life. The results of this study can be used to develop phase-specific, patient-centered, and tailored interventions for living kidney donors.

• Archives of Plastic Surgery
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• v.39 no.2
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• pp.126-129
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• 2012

Background : Facial capillary malformations (CMs) rarely recede; they often become darker and raised in proportion to their growth. These malformations may hypertrophy in adulthood, resulting in increased disfigurement and dysfunction. Laser treatment is considered a first-line therapy for focal CMs, but thick wide lesions, which are accompanied by hypertrophy and have a well-circumscribed nodularity, may be treated with surgical excision and reconstruction. Methods : We retrospectively reviewed the records of 25 consecutive patients who had undergone complete or partial excisions of facial capillary malformations in our unit. After the excisions, the defects that encompassed their facial aesthetic units were subsequently covered by various methods, including primary closures, local flaps, expanded flaps, split-thickness skin grafts, and full thickness skin grafts. Results : The data demonstrated satisfactory results and reliability. Our patients were treated without significant complications, and all of the patients were moderately or fully satisfied with the outcome of their surgeries. Conclusions : Among the many reconstructive options for adult patients with facial capillary malformations, thick split-thickness skin grafts can be a good choice for the coverage of widely excised wounds.