Dyskeratosis congenita is a rare genodermatosis of ectodermal dysplasia, which is characterized by the diagnostic triad consisting of reticulated hyperpigmentation, dystrophic nails, and leukoplakia. There is a predisposition to malignancy, particularly at sites of leukoplakia. Bone marrow failure can occur in about a half of the cases. A 16-year-old boy was presented with asymptomatic reticulated pigmentation of the neck and nail dystrophy. The patient also had leukoplakia on the tongue, nasolacrimal duct obstruction and cataract. The histopathological findings taken from the reticulated lesion were consistent with poikiloderma atrophicans vasculare. These clinical and histopathological findings were typical features of dyskeratosis congenita.
Adolescent ; Bone Marrow ; Cataract ; Dyskeratosis Congenita* ; Ectodermal Dysplasia ; Humans ; Hyperpigmentation ; Leukoplakia ; Male ; Nasolacrimal Duct ; Neck ; Pigmentation ; Tongue

PURPOSE: The occurrence of traumatic aneurysm is rare in head injury, but this complication is important as it is a potentially treatable cause delayed onset of intracranial hemorrhage. Authors report one case of traumatic aneurysm involving A1 and A2 junction of anterior cerebral artery. MATERIALS AND METHODS: A-28-year-old man with traffic accident was examined with brain CT & cerebral angiography. RESULTS: l) Brain CT:lnitial scan shows multiple skull fractures involving right frontal bones with subarachnoid hemorrhage and pneumocephalus. Follow-up scan shows intracerebral hemorrhage at bilateral frontal lobes. 2) Cerebral angiography:A traumatic aneurysm which is slowly filling and delayed emptying is noted at the junctional portion of A1 and A2 segment of the anterior cerebral artery. CONCLUSION: This report demonstrates radiologic findings of traumatic aneurysm at anterior cerebral artery with the brief review of the literatures.
Accidents, Traffic ; Aneurysm ; Anterior Cerebral Artery ; Brain ; Cerebral Angiography ; Cerebral Hemorrhage ; Craniocerebral Trauma ; Follow-Up Studies ; Frontal Bone ; Frontal Lobe ; Intracranial Aneurysm* ; Intracranial Hemorrhages ; Pneumocephalus ; Skull Fractures ; Subarachnoid Hemorrhage

PURPOSE: The purposes of this study were: 1) To describe the level of awareness of patient safety culture and nursing care activities for patient safety and, 2) To identify factors affecting nursing care activities for patient safety among oncology nurses working at inpatient care units. METHODS: The study sample included 221 nurses working in oncology wards. After obtaining IRB approval, data collection was done with structured self-administered questionnaires, from September 13 to October 13, 2013. Data analysis was done using SPSS WIN 18.0. RESULTS: The mean score of awareness of patient safety culture was 3.46 and the mean score of nursing care activities for patient safety was 4.31 both on a scale of 5.0. Significant factors affecting nursing care activities for patient safety were the open attitude of the supervisor toward patient safety (beta=.19, p=.005) and the degree of patient safety within a ward perceived by the oncology ward nurses (beta=.23, p=.003). These factors explained 31% of the subjects' nursing care activities for patient safety (F=6.08, p<.001). CONCLUSION: To increase nursing care activities for patient safety in the oncology ward, a strategy such as an education program for increasing the awareness of patient safety is needed.
Data Collection ; Education ; Ethics Committees, Research ; Humans ; Inpatients ; Nursing Care* ; Oncology Nursing ; Patient Safety* ; Safety Management ; Statistics as Topic ; Surveys and Questionnaires

Purpose: This study aimed to evaluate the validity and reliability of the Korean version of the Quality of Life in LifeThreatening Illness - Family Carer Version 3 (QOLLTI-F v3) among Korean family caregivers of patients with terminal cancers. Methods: A total of 198 family caregivers of terminal cancer patients were investigated using the Korean version of QOLLTI-F v3. Its psychometric properties were examined in terms of factor structure, convergent validity, discriminant validity, criterion validity and internal consistency. Results: The convergent validity of the Korean version of QOLLTI-F v3 showed that the standardized regression weights for each item were above .50 except for two out of 16 items, the average variance extracted was above .50 for four out of five factors, and the critical ratio ranged from .50 to .85. The discriminant validity of the items across all factors was confirmed. The value for Cronbach’s α of 16 items was .84. Conclusion The Korean version of the QOLLTI-F v3 is a reliable instrument with acceptable convergent and high discriminant validity for measuring the quality of life of families of people with life-threatening illnesses, including families of people with terminal cancers.

Nail-patella syndrome is a relatively rare autosomal dominant disorder characterized by dysplastic nail, hypoplastic or absent patella, and dislocation of radial head and iliac horns. In addition, renal abnormalities have been reported. The usual clinical signs of the renal involvement are asymptomatic proteinuria, microscopic hematuria, and in some cases progression to end stage renal disease. We present the case of adult with nail-patella syndrome, who developed proteinuria. Electron microscopy revealed irregular thickening of the glomerular basement membrane with areas of rarefaction, giving rise to a pathognomonic "moth-eaten" appearance.
Adult ; Animals ; Dislocations ; Glomerular Basement Membrane ; Head ; Hematuria ; Horns ; Humans ; Kidney Failure, Chronic ; Microscopy, Electron ; Nail-Patella Syndrome* ; Patella ; Proteinuria

Cutaneous focal mucinosis is a condition with an asymptomatic, small, dermal, white to flesh-colored papule with a predilection for the face, neck, trunk, and extremities. This is usually presented as solitary lesion but rarely as multiple papules or nodules. Histologically, a localized area of the collagen in the upper dermis is largely replaced by homogenous mucinous material in which spindle-shaped fibroblasts are scattered. We report a case of cutaneous focal mucinosis which was presented as multiple lesions covering widespread skin lesion throught the face, chest and back.
Collagen ; Dermis ; Extremities ; Fibroblasts ; Mucinoses* ; Mucins ; Neck ; Skin* ; Thorax

The clinical and pathological features of warty dyskeratoma are presented. Warty dyskeratoma is a rarely encountered, solitary, benign cutaneous tumor occurring most often on the head and neck and to our knowledge, no cases associated with cutaneous horn have been published in western literature. We experienced a case of warty dyskeratoma with a cutaneous horn occurring in a 70-year-old male, who had a 1.3 cm-sized and slowly growing nodule on his neck. A brief review of the literature, was made especially in relation to the pathological findings and histogenesis of warty dyskeratoma.
Aged ; Animals ; Head ; Horns* ; Humans ; Male ; Neck

PURPOSE: This study was done to explore experiences of persons living through the periods of cancer diagnosis, treatment, and self-care. METHODS: With permission, texts of 29 cancer survival narratives (8 men and 21 women, winners in contests sponsored by two institutes), were analyzed using Kang's Korean-Computerized-Text-Analysis-Program where the commonly used Korean-Morphological-Analyzer and the 21st-century-Sejong-Modern-Korean-Corpora representing laymen's Korean-language-use are connected. Experiences were explored based on words included in 100 highly-used-morphemes. For interpretation, we used 'categorizing words by meaning', 'comparing use-rate by periods and to the 21st-century-Sejong-Modern-Korean-Corpora', and highly-used-morphemes that appeared only in a specific period. RESULTS: The most highly-used-word-morpheme was first-person-pronouns followed by, diagnosis.treatment-related-words, mind-expression-words, cancer, persons-in-meaningful-interaction, living and eating, information-related-verbs, emotion-expression-words, with 240 to 0.8 times for layman use-rate. 'Diagnosis-process', 'cancer-thought', 'things-to-come-after-diagnosis', 'physician.husband', 'result-related-information', 'meaningful-things before diagnosis-period', and 'locus-of-cause' dominated the life of the diagnosis-period. 'Treatment', 'unreliable-body', 'husband . people . mother . physician', 'treatment-related-uncertainty', 'hard-time', and 'waiting-time represented experiences in the treatment-period. Themes of living in the self-care-period were complex and included 'living-as-a-human', 'self-managing-of-diseased-body', 'positive-emotion', and 'connecting past . present . future'. CONCLUSION: The results show that the experience of living for persons with cancer is influenced by each period's own situational-characteristics. Experiences of the diagnosis and treatment-period are negative disease-oriented while that of the self-care period is positive present-oriented.
Family Relations ; Female ; Humans ; Male ; Neoplasms/diagnosis/*psychology/therapy ; Patient Acceptance of Health Care ; Professional-Patient Relations ; Program Development ; Self Care ; User-Computer Interface

Nasal osteotomy for aesthetic results is required in patients who have broad nasal bone. Since the procedure is difficult, many operators hesitate the surgery. From January 2007 to July 2009, we performed 77 cases of nasal osteotomy, consisting of paramedian oblique medial osteotomy or median oblique-medial osteotomy and percutaneous lateral osteotomy. Most of the patients had satisfying results, however three patients had asymmetric shape and inappropriate inward movement of nasal bone which required revision with percutaneous lateral osteotomy. Osteotomy procedure varies, depending on surgeon's preference and experience, however, each surgeon should be aware of good and weak points of those procedures and use the most suitable method. In order to achieve maximal satisfying results, preoperative analysis of patients and minimal invasive handling should be done. Based on these efforts, this method could broadly be used in the aesthetic field.
Handling (Psychology) ; Humans ; Nasal Bone ; Nose ; Osteotomy ; Rhinoplasty

Purpose: Nurses’ job satisfaction corresponds with personal intrinsic value, and is the strongest predicting turnover and job retention intention. This study identified the effectiveness of job satisfaction interventions related to reinforcing intrinsic motivation (JSI-RIM) for hospital nurses. Methods: This study used four core non-Korean databases (Cochrane Library, CHINHL, EMBASE, PubMed), and five Korean databases to search for RCT and NRCT articles published in English and Korean from inception to June 2019. Meta-analysis was performed using the RevMan 5.3.5 program. Results: Thirteen studies featuring 645 hospital nurses were selected for final analysis.A significant large effect was noted on self-efficacy. The effect size on perceived stress and job satisfaction were moderate; however, the effect on resilience outcomes was not significant. Conclusion This study generated scientific evidence that would facilitate efficient job adaptation for nurses. Additionally, intrinsic factors, including job identity and meaning of work, need to be included in JSI-RIM.