PURPOSE: We report two cases of Cohen syndrome associated with ophthalmologic findings, which is the first such report from Korea. METHODS: A 14-year-old boy and a 13-year-old girl, a brother and sister, who had visited the department of pediatrics for evaluation of developmental disorders was transferred to the department of ophthalmology due to evidence of nyctalopia and decreased visual acuity. There, refraction, ultrasonography, and fundus examinations were performed. RESULTS: The children had high myopia, and refractive astigmatism. Bull's eye maculopathy with a pale disc was found on the fundus examination. There were characteristic facial appearances including wave-shaped, down-slanting palpebral fissures and short philtrum. They also had grimacing expressions upon smiling. Neutropenia was identified in the female patient. CONCLUSIONS: Cohen syndrome should be suspected and considered in the differential diagnosis of patients with high myopia, nyctalopia, and decreased visual acuity.
Adolescent ; Astigmatism ; Child ; Diagnosis, Differential ; Female ; Humans ; Korea ; Lip ; Male ; Myopia ; Neutropenia ; Night Blindness ; Ophthalmology ; Pediatrics ; Siblings ; Smiling ; Ultrasonography ; Visual Acuity

Congenital renal arteriovenous malformation is a rare lesion. Nephrectomy or partial nephrectomy is a well-known treatment and as alternative method, transcatheter embolization therapy has been developed. We report a case of congenital arteriovenous malformation manifested by intermittent right flank pain and gross hematuria with blood clots, cured by means of transcatheter embolization therapy during angiographic study with polyvinyl alcohol foam (IVALON).
Arteriovenous Malformations* ; Flank Pain ; Hematuria ; Kidney ; Nephrectomy ; Polyvinyl Alcohol

The vaginal stone associated with vesico-vaginal fistula is quite rare and its cause, frequency, composition and others are poorly understood. We report a case of huge vaginal stone associated with vesico-vaginal fistula in 57 years old woman. considering of the possible causes of stone formation in the vagina and its composition, prevention, treatment complication and others. A stone, 6 x 7 x 5cm in size, was removed by litholapaxy. The stone was composed of uric acid, magnesium ammonium phosphate and carbonate appetite.
Ammonium Compounds ; Appetite ; Carbon ; Female ; Fistula* ; Humans ; Lithotripsy ; Magnesium ; Middle Aged ; Uric Acid ; Vagina ; Vesicovaginal Fistula

Fibromuscular dysplasia is a nonatheromatous, noninflammatory arterial disorder that results in stenosis and/or aneurysm formation and rarely involves the mesenteric arteries. Herein, we report a case of fibromuscular dysplasia limited to the inferior mesenteric artery, which manifested with arterial aneurysms and ischemic colitis.

Serous cystic neoplasms of the pancreas are usually benign, with a low malignant potential. Herein, we report a case of malignant serous cystic neoplasm of the pancreas treated with subtotal pancreatectomy, which progressed to local recurrence and metachronous hepatic metastasis during the regular follow-up period.

Serous cystic neoplasms of the pancreas are usually benign, with a low malignant potential. Herein, we report a case of malignant serous cystic neoplasm of the pancreas treated with subtotal pancreatectomy, which progressed to local recurrence and metachronous hepatic metastasis during the regular follow-up period.

PURPOSE: To explore the effects of stimulus duration, stimulus intensity, and background luminance on the amplitude and waveform of the ON- and OFF- responses of photopic ERG and to provide standard parameters of ON- and OFF- responses for normal Korean populations. METHODS: Twenty normal subjects (20 eyes) were enrolled to record photopic ON-and OFF-responses using a contact lens electrode with built-in LEDs and an LED-driver. The influence of stimulus duration on wave amplitudes was studied at flash durations that varied from 5 to 200 ms. The influence of stimulus intensity was studied with 0.4, 0.7, 1.1, 1.4, 1.7, 1.9, 2.0, and 2.1 log cd/m2. In addition, the influence of background luminance was studied with 20, 30, 40, and 50 cd/m2. RESULTS: Among 20 normal subjects, the d-waves in 16 subjects were isolated from b-waves with more than 50 ms of stimulus duration. The d-wave was observed for a 30-ms stimulus duration in 3 subjects and for a 100-ms duration in 1 subject. The amplitudes of the b-and d-waves increased as stimulus intensity was increased. The amplitudes of b-and d-waves decreased as background luminance was increased. CONCLUSIONS: Our results suggest that the clinical use of ON-and OFF-response recording can be obtained with stimulus parameters from 100 to 150 ms in duration and a 1.7-2.1 log cd/m2 intensity under 30-40 cd/m2 background luminance.
Electrodes ; Electroretinography

The aims of this study were to develop strategies and algorithms of calculating food commodity intake suitable for exposure assessment of residual chemicals by using the food intake database of Korea National Health and Nutrition Examination Survey (KNHANES). In this study, apples and their processed food products were chosen as a model food for accurate calculation of food commodity intakes uthrough the recently developed Korea food commodity intake calculation (KFCIC) software. The average daily intakes of total apples in Korea Health Statistics were 29.60 g in 2008, 32.40 g in 2009, 34.30 g in 2010, 28.10 g in 2011, and 24.60 g in 2012. The average daily intakes of apples by KFCIC software was 2.65 g higher than that by Korea Health Statistics. The food intake data in Korea Health Statistics might have less reflected the intake of apples from mixed and processed foods than KFCIC software has. These results can affect outcome of risk assessment for residual chemicals in foods. Therefore, the accurate estimation of the average daily intake of food commodities is very important, and more data for food intakes and recipes have to be applied to improve the quality of data. Nevertheless, this study can contribute to the predictive estimation of exposure to possible residual chemicals and subsequent analysis for their potential risks.
Eating ; Korea* ; Malus ; Nutrition Surveys* ; Risk Assessment

PURPOSE: The death of retinal pigment epithelial(RPE) cells plays an important role in the development of age-related macular degeneration. We studied whether ultraviolet could induce apoptotic cell death and the increase of reactive oxygen intermediates (ROI) in the RPE cells. METHODS: Cultured human RPE cells were exposed to doses of between 0 and 80 mJ/cm2 UVB as an attached monolayer. The number of viable cells was assessed by trypan blue assay. The apoptosis was determined by flow cytometry using annexin V and propidium iodide (PI). Intracellular ROI was measured using DCFH-DA. RESULTS: UVB-exposed cells showed a dose-dependent cell death. The number of cells decreased in 20~30% at the dose of 40 mJ/cm2 UVB 24 hours after UVB exposure. The proportion of annexin V positive cells and intracellular ROI increased after UVB exposure. CONCLUSIONS: These results suggest that UV-induced oxidative damage may be one of the important mechanisms of RPE cell death.
Annexin A5 ; Apoptosis ; Cell Death ; Epithelial Cells* ; Flow Cytometry ; Humans ; Macular Degeneration ; Oxygen ; Propidium ; Retinal Pigment Epithelium ; Retinaldehyde* ; Trypan Blue