Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

PURPOSE: The purpose of this study was to explore the experience of hope of terminal cancer patients in Korea. METHODS: Seven participants receiving hospice care were interviewed about hope experiences based on Parse's research methodology. RESULTS: Practical propositions derived from the structure of the hope experience were: Terminal cancer patients are beings newly recognizing the value of their lives by maintaining their hopes while they are experiencing powerlessness due to feelings of loss and distress. Ways of truly being with them are accepting their experience of distress, understanding the existence of paradoxical relationships, active symptom management, and finding hope in the current situation. Through these processes, the patients restructure their remaining lives for peaceful death by changing their perceptions about death. This structure can be interpreted as 'the hope experience of terminal cancer patients is a process of changing the value of life, overcoming fear of death, enhancing meaning, and transcending.' CONCLUSION: The concept of 'true presence' applied in this study was proved to be an effective nursing practice improving quality of life of the patients. Parse's Human Becoming Methodology may be applied to terminal cancer patients in the future.

PURPOSE: To determine impacts of symptom clusters, performance and emotional status on the quality of life of gynecologic cancer patients. METHODS: Subjects completed questionnaires consisting of four measurements: symptom cluster, performance and emotional status, and the quality of life. A total of 104 completed data sets were analyzed by descriptive statistics, t-test, ANOVA, Pearson's correlation coefficient, and a multiple regression analysis using the SPSS 21.0 program. RESULTS: Fatigue was identified as the most prevalent symptom (77.9%) and sweating (2.08) as the most severe and uncomfortable symptom (1.80). Six symptom clusters, performance status, anxiety and depression were negatively correlated with quality of life. Four symptom clusters were positively correlated with performance status, and six symptom clusters were positively correlated with anxiety and depression. Factors affecting quality of life were abdominal discomfort cluster (β=−0.23, p=0.005), performance status (β=−0.20, p=0.020), and depression (β=−0.42, p < 0.001). The model was statistically significant explaining 42.5% of variance (F=26.369, p < 0.001). CONCLUSION: The findings supported that symptom clusters and depression negatively influence the quality of life and need to be addressed as we are caring for patients and promoting quality of life.
Anxiety ; Dataset ; Depression ; Fatigue ; Humans ; Quality of Life ; Sweat ; Sweating

PURPOSE: To report a case of a patient who underwent penetrating keratoplasty due to acute hydrops six years after undergoing a bilateral LASIK procedure. METHODS: A 19-year old male patient with -2.75 diopter myopia received a bilateral LASIK operation at a local clinic. Forty-seven months later, -2.00 cyl axis 130o astigmatism was induced in his left eye and additional surgery was performed to correct the LASIK-induced astigmatism. The patient was referred to our hospital 16 months after the secondary operation due to reduced visual acuity in his left eye. He was diagnosed with keratectasia and acute hydrops, and penetrating keratoplasty was performed. RESULTS: The retrospective analysis of the corneal topography prior to the LASIK surgery revealed that the inferior minus superior(I-S) value was 1.25 in the right eye and 1.3 in the left eye. At the time of the first evaluation at out hospital, a slit lamp examination showed an approximate 8 x 7.5mm sized area filled with aqueous humor between the corneal flap and residual stroma, and Descemet's membrane rupture was observed. Furthermore, the histopathological section of the patient's cornea confirmed keratocytes with myxoid degeneration and edematous change. CONCLUSIONS: In young patient, particularly those with suspicious asymptomatic keratoconus, LASIK and further enhancement surgery afterwards should be considered and approached in a cautious and deliberate manner.
Male ; Humans

BACKGROUND: Non-small cell lung cancer is one of the most frequent cause of death due to cancer in men, and its incidence among women is rapidly increasing. Although there has been a recent surge of interest in combined modality therapy for stage III non-small cell lung cancer(NSCLC), the optimal treatment is still not well established. Thoracic irradiation has long been the gold standard for locally advanced unresectable NSCLC. However, although conventional radiotherapy(XRT) can palliate symptom and improve local control of disease, it huts at most only a modest effect on survival. Recently, cisplatin(cia-diamminedichloroplatinum ) has been reported to enhance the cell-killing effect of radiation For patients with unresectable NSCLC, cisplatin-based concurrent chemoradiotherapy(CCRT) had the advantage of therapeutic response over XRT alone and therapeutic side effect more commonly occurred in CCRT group in EORTC(European Organization for Research and Treatment of Cancer) and other trials. Objectives : We compared therapeutic response, compliance, and side effects between CCRT and XRT in patients with advanced NSCLC. Patients and METHOD: Thirty patients with biopsy-proven inoperable NSCLC were randomized to one of two treatment arms. Arm A consisted of XRT, radiotherapy for 4~6 weeks(1.8 Gy given 20~33 times, in five fractions a week), and arm B consisted of CCRT, radiotherapy for 2 weeks(3 Gy given 10 times, in five fractions a week), followed by 3 week rest period and then radiotherapy 2 more weeks(2.5 Gy given 10 timed in five fractions a week), combined with 6mg cisplatin per square meter, given daily before radiotherapy. We evaluate therapeutic response, compliance, change of performance status, side effects, and radiation pneumonitis by using the author's made scoring system. RESULTS: There was no significant difference in therapeutic response and compliance. But there was a significantly lower laboratory complication and radiation pneumonitis in CCRT group (p<0.05). There's significant negative correlation between stage and therapeutic response score in both groups(R=0.353, p<0.05). In both groups, patients with squamous cell carcinoma had a tendency to higher therapeutic response score than those with adenocarcinoma. CONCLUSION: There was. no difference between CCRT and XRT in respect to therapeutic response and compliance. But CCRT had a advantage of decreased side effects.
Adenocarcinoma ; Arm ; Carcinoma, Non-Small-Cell Lung ; Carcinoma, Squamous Cell ; Cause of Death ; Chemoradiotherapy* ; Cisplatin ; Combined Modality Therapy ; Compliance ; Female ; Humans ; Incidence ; Lung Neoplasms* ; Lung* ; Male ; Radiation Pneumonitis ; Radiotherapy*

Primary tracheal tumors, especially lipoma, are very rare. Symptoms of tracheal tumor mimic common upper airway diseases. It is important that they may initially be misdiagnosi4 to bronchial asthma, chronic bronchitis, resulting in a delay in diagnosis. We report a case of tracheal lipoma who was found 6 months after first noticing symptoms including dyspnea, foreign body sensation, successfully removed by bronchoscopic polypectomy.
Asthma ; Bronchitis, Chronic ; Diagnosis ; Dyspnea ; Foreign Bodies ; Lipoma* ; Sensation ; Trachea

Transplanting fetal kidney cells (FKCs) can regenerate kidney. This requires in vitro expansion in cell number to acquire enough cells for transplantation. However, FKCs may change their cellular characteristics during expansion and, thus, may not regenerate kidney tissue upon transplantation. We investigated how cell culture period affects cellular characteristics and in vivo regenerative potential of FKCs. As the passage number increased, cell growth rate and colony forming ability decreased while senescence and apoptosis increased. To examine in vivo regenerative potential, FKCs cultured through different numbers of passages were implanted into the parenchyma of kidneys of immunodeficient mice using fibrin gel for 4 wk. Histological analyses showed passage-dependent kidney tissue regeneration, and the regeneration was better when cells from lower number of passages were implanted. This result shows that in vitro culture of FKCs significantly affects the cell characteristics and in vivo tissue regenerative potential.
Animals ; Apoptosis/physiology ; Cell Aging/physiology ; Cell Culture Techniques ; Cell Proliferation ; Cells, Cultured ; Colony-Forming Units Assay ; Female ; Fetal Tissue Transplantation/methods/physiology ; Fetus/cytology/*physiology ; Kidney/embryology/*physiology ; Mice ; Mice, Inbred BALB C ; Mice, Nude ; Rats ; Rats, Sprague-Dawley ; Regeneration/*physiology

PURPOSE: To determine whether insulin-like growth factor (IGF-1) affects transforming growth factor (TGF-beta)- mediated fibronectin accumulation in human lens epithelial cell line (HLE B-3) cells. MATERIALS AND METHODS: HLE B-3 cells were incubated for 24 hours with TGF-beta (10ng/ mL), IGF-1 (10ng/mL), or both. Expression of the fibronectin gene was determined using a real-time reverse transcriptase-polymerase chain reaction (RT-PCR). Fibronectin levels were examined using western blot analysis and immunofluorescence staining. RESULTS: Expression of the fibronectin gene was not different between the TGF-beta/IGF-1 treated group and the TGF-beta treated group (p= 0.116). However, western blot analysis demonstrated decreased fibronectin levels in human lens epithelial cells treated with TGF-beta and IGF-1 compared to those treated with TGF-beta only (p < 0.01). Immunofluorescence staining disclosed inhibition of TGF-beta-induced fibronectin in the presence of IGF-1. CONCLUSION: This study suggests that IGF-1 counteracts TGF-beta-mediated fibronectin accumulation in human lens epithelial cells.
Cell Line ; Epithelial Cells/cytology/*drug effects/metabolism ; Fibronectins/*genetics/metabolism ; Fluorescent Antibody Technique ; Gene Expression Regulation/drug effects ; Humans ; Insulin-Like Growth Factor I/*pharmacology ; Lens, Crystalline/cytology ; Reverse Transcriptase Polymerase Chain Reaction ; Transforming Growth Factor beta/*pharmacology

PURPOSE: We performed invasive thermometry to verify the elevation of local temperature in the liver during hyperthermia. MATERIALS AND METHODS: Three 40-kg pigs were used for the experiments. Under general anesthesia with ultrasonography guidance, two glass fiber-optic sensors were placed in the liver, and one was placed in the peritoneal cavity in front of the liver. Another sensor was placed on the skin surface to assess superficial cooling. Six sessions of hyperthermia were delivered using the Celsius TCS electro-hyperthermia system. The energy delivered was increased from 240 kJ to 507 kJ during the 60-minute sessions. The inter-session cooling periods were at least 30 minutes. The temperature was recorded every 5 minutes by the four sensors during hyperthermia, and the increased temperatures recorded during the consecutive sessions were analyzed. RESULTS: As the animals were anesthetized, the baseline temperature at the start of each session decreased by 1.3degrees C to 2.8degrees C (median, 2.1degrees C). The mean increases in temperature measured by the intrahepatic sensors were 2.42degrees C (95% confidence interval [CI], 1.70-3.13) and 2.67degrees C (95% CI, 2.05-3.28) during the fifth and sixth sessions, respectively. The corresponding values for the intraperitoneal sensor were 2.10degrees C (95% CI, 0.71-3.49) and 2.87degrees C (1.13-4.43), respectively. Conversely, the skin temperature was not increased but rather decreased according to application of the cooling system. CONCLUSION: We observed mean 2.67degrees C and 2.87degrees C increases in temperature at the liver and peritoneal cavity, respectively, during hyperthermia. In vivo real-time thermometry is useful for directly measuring internal temperature during hyperthermia.
Anesthesia, General ; Animals ; Fever* ; Glass ; Liver* ; Peritoneal Cavity ; Skin ; Skin Temperature ; Swine ; Thermometry ; Ultrasonography

PURPOSE: This study examined the effects of aerobic exercise using a flex band on the improvement of physical functions & body image in breast cancer women undergoing radiation therapy after a mastectomy. METHOD: Women with breast cancer(n = 26) were assigned to an experimental group(EG, n = 15) and control group(CG, n = 11). The E.G. participated in an aerobic exercise program with a 60% to 80% intensity of maximal heart rate for 25 minutes during the main exercise, 3 times per week, for 6 weeks. The EG did not exercise regularly for 3 months before participating in this program. The CG received no exercise treatment during the research period. Data were analyzed using the chi-square-test and Mann-Whitney U test by the SPSS version 11.0 program at a 5% significant level. RESULTS: Group analysis revealed that the EGwomen had significantly more improved cardiopulmonary functions, ROM of the affected shoulder joint, and body image compared to the CG. CONCLUSION: Aerobic exercise using a flex band may be an effective rehabilitative measure for mastectomy women with respect to cardio-pulmonary functions, ROM, & body image. Further studies are recommended to study early rehabilitation programs within 10 days post-operatively.
Adult ; *Body Image ; Breast Neoplasms/radiotherapy/*rehabilitation/surgery ; Combined Modality Therapy ; Female ; Heart Rate ; Humans ; *Mastectomy ; Middle Aged ; *Muscle Stretching Exercises/instrumentation ; Oximetry ; Program Evaluation ; *Range of Motion, Articular