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

No abstract available.
Granulomatous Disease, Chronic* ; Trimethoprim-Sulfamethoxazole Combination

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

PURPOSE: To classify Avellino corneal dystrophy (ACD) into 3 groups by slit lamp photography and to evaluate the inter-group differences. METHODS: DNA analysis was performed in one member from each of 45 families who were clinically diagnosed as ACD. Corneal opacities were classified into mild (group I), moderate (group II), and severe (group III) based on slit lamp photograph. RESULTS: Six patients were confirmed as homozygous ACD and ninety-two as heterozygous ACD. One homozygous patient was revealed to have a novel phenotype. The age increased with increasing severity, and the proportion of females in group III was significantly higher than that of the other two groups (p=0.001). The prevalence of laser in situ keratomileusis (LASIK) increased in group III (p=0.001), and the incidence of pterygium increased in group I compared with the other two groups (p=0.025). CONCLUSIONS: ACD was aggravated with age, while pterygium and LASIK altered the natural course of ACD.
Classification* ; Corneal Opacity ; DNA ; Female ; Humans ; Incidence ; Keratomileusis, Laser In Situ ; Phenotype ; Photography ; Prevalence ; Pterygium

Purpose: To compare the visual outcomes and corneal aberrations between wavefront-optimized (WFO) and corneal wavefront-guided (WFG) photorefractive keratectomy (PRK) in low to moderate myopia. Methods: Twenty-seven eyes treated with WFO and 29 eyes treated with WFG PRK using a Schwind Amaris 750S Excimer laser were included after 6 months of postoperative follow-up. Uncorrected distance visual acuity, corrected distance visual acuity, refractive errors, corneal higher-order aberrations (HOA) and corneal thickness obtained using a Scheimpflug system, and central ablation depth and volume were evaluated during the preoperative period and again at the postoperative 6-month visits. Results: Postoperatively, uncorrected distance visual acuity, corrected distance visual acuity, manifest spherical equivalent, and refractive astigmatism were improved in both groups, and there was no statistically significant difference between the two groups. There was no significant difference in safety, efficacy, or predictability of the refractive outcome. Postoperative total corneal HOA root mean square (RMS), coma RMS, and spherical aberration were significantly increased in both groups. Among these, only spherical aberration showed a significant difference between the two groups, with greater increase in the WFO group at 6 months postoperatively. The changes in corneal HOA RMS and spherical aberration were smaller in the WFG group, and this benefit was marked in eyes with high HOA RMS (≥0.4 μm) and spherical aberration (≥0.2 μm). Even though ablation volume in the WFG group was much larger than that of the WFO group, there was no significant difference in postoperative central and peripheral corneal thickness between the two groups. Conclusions Both WFO and WFG PRK using a Schwind Amaris 750S laser for low to moderate myopia were safe and effective at improving visual and refractive outcomes. However, WFG PRK induced fewer spherical aberrations than WFO PRK and may be more advantageous for eyes with high HOA root mean square or spherical aberration.

Adenocarcinoma of small intestine is rare disease, but its incidence of iluem is the least of small intestinal adenocarcinoma. Its rarity and vague symptoms results in delayed diagnosis, treatment and poor prognosis, Recently we experienced a 65 years old man of ileal adenocarcinoma with liver metastasis, which does not obstuct intestinal lumen, We treat the metastatic tumor with chemoembolization(lipiodol and doxorubicin) through the hepatic artery and so we report the case with review of literature.
Adenocarcinoma ; Aged ; Delayed Diagnosis ; Hepatic Artery ; Humans ; Incidence ; Intestine, Small ; Liver ; Neoplasm Metastasis* ; Prognosis ; Rare Diseases

No abstract available.
Reflex Sympathetic Dystrophy* ; Reflex*

PURPOSE: The purpose of this study was to analyze the research papers published in three nursing journals to suggest the direction for Journal of Korean Oncology Nursing (JKON). METHODS: To compare JKON with Journal of Korean Academic Society of Nursing Education and Cancer Nursing, all the research papers published in those three journals, 2010 were reviewed using an analysis criteria developed by the researchers, focusing on type of research, characteristics of authors and subjects, research design, data collection and analysis methods, sample size estimation, and ethical considerations regarding data collection. RESULTS: JKON lacked research papers which were supported by research funds, produced by multidisciplinary teams, addressing cancer survivors or patients with metastatic cancers, and written in qualitative methodologies. However, JKON showed higher ratio of research papers than the other two journals which were adapted from thesis or dissertations, describing sample size estimation process precisely, and participating subjects diagnosed with various cancers. CONCLUSION: The study found out that JKON is presenting well the area of oncology nursing in Korea and also has several weak points that need to be improved. The study therefore suggested several recommendations for the JKON to take the professional and global leader roles.
Data Collection ; Education, Nursing ; Financial Management ; Humans ; Korea ; Manuscripts as Topic ; Nursing Research ; Research Design ; Research Subjects ; Sample Size ; Survivors

PURPOSE: To describe psychosocial adjustment of low-income Koreans who have cancer. METHODS: Data were collected during 2008 using individual in-depth interviews with 18 Korean people with cancer. The income status of the participants was low, 11 were recipients of the National Basic Livelihood Protection program. Mean age was 58.3 yr and 11 were female. Five participants had stomach cancer, five, colorectal cancer, and four, breast cancer. Data were analyzed using grounded theory methodology. RESULTS: The core category emerged as 'bearing up alone with double suffering'. 'Poverty and cancer: A double suffering' emerged as a causal condition. The adjustment process consisted of three stages: 'forming a treatment will to live' ,'practicing for the cure',and 'restructuring self and repaying favors'. Each stage indicated action-interaction strategies which were employed to bear up alone with double suffering during the illness process. Self-reflection, parental responsibility, and support from the public sector played important roles in overcoming the double suffering. Two types of consequences were identified: Transcended life and strained life suppressed by poverty and cancer. CONCLUSION: The results provide insights into the psychosocial adjustment process for low income Korean with cancer and can be used in developing and implementing efficient home-care services for these people.
*Adaptation, Psychological ; Aged ; Depression ; Female ; Humans ; Interviews as Topic ; Male ; Middle Aged ; Neoplasms/*psychology ; Poverty ; Republic of Korea ; Social Support ; Stress, Psychological

PURPOSE: To evaluate tear eosinophil cationic protein (ECP) as a severity marker for atopic keratoconjunctivitis (AKC) and seasonal/perennial allergic conjunctivitis (SAC/PAC). METHODS: Tear ECP levels were measured by chemiluminescent immunometric assay in 7 eyes of 7 patients with AKC, 13 eyes of 13 patients with SAC/PAC, and 10 eyes of 10 healthy control subjects. All AKC and SAC/PAC patients underwent conjunctival injection and papillary formation grading. Tear ECP levels were investigated with reference to the clinical parameters of allergic conjunctivitis (papillary formation and conjunctival injection scoring). RESULTS: Tear ECP levels in patients with AKC were significantly higher than those in patients with SAC/PAC and in control subjects (p = 0.012 and p = 0.003, respectively). The number of patients with papillary formation scores of 2-3 was significantly higher in the AKC group than in the SAC/PAC group (p = 0.016). The number of patients with conjunctival injection scores of 2-3 did not significantly differ between the AKC and SAC/PAC groups (p = 0.128). All AKC patients obtained papillary formation scores of 2-3, and tear ECP levels in patients with conjunctival injection scores of 2-3 were significantly higher than in patients with scores of 0-1 in the AKC group (p < 0.001). In the SAC/PAC group, tear ECP levels in patients with papillary formation scores of 2-3 were significantly higher than in patients with scores of 0-1 (p = 0.046). CONCLUSIONS: This study suggests that tear ECP was a useful marker to diagnose and assess the severity of disease in patients with AKC as well as SAC/PAC. It would be useful to monitor therapeutic outcome in allergic conjunctivitis.
Conjunctivitis, Allergic ; Eosinophil Cationic Protein* ; Humans ; Keratoconjunctivitis*