Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait spots and neurofibrobmas, or only neurofibroma, limited to one region of the body. Disease-associated systemic involvement is uncommon. Most patients with segmental neurofibromatosis do not have a family history of neurofibromatosis. In Korea, there have been 3 reported cases of segmental neurofibromatosis. None of them had caf6-au-fait spots, systemic involvement, or family history. This report describes a case of segmental neurofibromatosis in a 25-year-old woman who had Becker's nevus-like cafe-au-lait spots and this was linked to the presence of Fanconi's syndrome in a second degree relative of the patient.
Adult ; Cafe-au-Lait Spots ; Female ; Humans ; Korea ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1

PURPOSE: Signet-ring cell carcinoma is rather invasive and infiltrative than other histologic types of gastric cancer. We evaluated the characteristic CT findings of signet-ring cell carcinoma especially in the intensity and pattern of contrast enhancement. MATERIALS AND METHODS: We analyzed the CT findings of 22 cases with histologically proven signet-ring cell carcinoma, and compared them with those of 35 cases with histologically proven tubular adenocarcinoma. RESULTS: The double ring enhancement of the gastric mass was seen in 12 cases of signet-ring cell carcinoma and only one case of tubular adenocarcinoma. The masses of signet-ring cell carcinoma were enhanced more by the CT number of 10.2 than those of tubular adenocarcinoma. Of the masses of signet-ring cell carcinoma, those showed double ring enhancement were more intensely enhanced than those showed diffuse enhancement by the CT number of 22.9. CONCLUSION: We thought that neovascularity and different infiltration of the tumor cells in the gastric wall were responsible for the intense enhancement and double ring sign of signet-ring cell carcinoma. The possiblity of signet-ring cell carcinoma is high if a gastric mass show double ring sign and strong contrast enhancement.
Adenocarcinoma ; Carcinoma, Signet Ring Cell* ; Stomach Neoplasms ; Stomach*

Prurigo pigmentosa is a distinct type of inflammatory dermatosis characterized by recurrent pruritic erythematous papules that resolve, leaving a peculiar, reticulate hyperpigmentation. Although the cause of prurigo pigmentosa is unknown, exogenous factors, such as friction from clothing, have been suggested as possible triggers in some cases. In other cases, however, systemic conditions such as diabetes mellitus, pregnancy, fasting, and dieting were associated with prurigo pigmentosa, suggesting involvement of endogenous factors in the pathogenesis. There have been reported cases of prurigo pigmentosa associated with ketosis. Because ketosis is commonly observed in association with fasting, dieting, and insulin dependent diabetes melitus, it may be involved in the pathogenesis of prurigo pigmentosa. Here, we describe a patient with prurigo pigmentosa associated with diabetes mellitus and ketosis. In this case, the prurigo pigmentosa improved with the disappearance of the ketosis, but the blood sugar levels did not correlate with the improvement.
Blood Glucose ; Clothing ; Diabetes Mellitus ; Diet ; Fasting ; Friction ; Humans ; Hyperpigmentation ; Insulin ; Ketosis* ; Pregnancy ; Prurigo* ; Skin Diseases

The Korea National Health and Nutrition Examination Survey (KNHANES) is a national program designed to assess the health and nutritional status of the noninstitutionalized population of South Korea. The KNHANES was initiated in 1998 and has been conducted annually since 2007. Starting in the latter half of 2008, ophthalmologic examinations were included in the survey in order to investigate the prevalence and risk factors of common eye diseases such as visual impairment, refractive errors, strabismus, blepharoptosis, cataract, pterygium, diabetic retinopathy, age-related macular degeneration, glaucoma, dry eye disease, and color vision deficiency. The measurements included in the ophthalmic questionnaire and examination methods were modified in the KNHANES IV, V, and VI. In this article, we provide detailed information about the methodology of the ophthalmic examinations in KNHANES in order to aid in further investigations related to major eye diseases in South Korea.
*Epidemiologic Methods ; Eye Diseases/*epidemiology ; Humans ; Nutrition Surveys/*statistics & numerical data ; Ophthalmology/*methods ; Prevalence ; Republic of Korea/epidemiology ; Risk Factors ; *Surveys and Questionnaires

BACKGROUND/AIMS: Neuroendocrine tumors (NET) of the pancreas are rare. Its prognosis is better than pancreas adenocarcinoma due to the slow growth, however, malignant NET of the pancreas are observed. The purposes of this study were to evaluate the clinical characteristics and to find the predictive factors of NET which are associated with malignancy and survival. METHODS: We retrospectively evaluated the clinical outcomes of 122 patients with NET of the pancreas who were pathologically diagnosed at Asan Medical Center between 1990 and 2006. RESULTS: Mean age of the patients was 48.9+/-14.0 years and there was no gender predilection. The major clinical manifestations were abdominal pain (44.0%) in non-functional tumor, neuroglycopenic symptoms (100%) in insulinoma and diarrhea (60%) in gastrinoma. Tumor size ranged from 4 to 140 mm (average 29.8+/-23.22). Ninety cases (73.8%) were classified as benign tumors and 32 cases (26.2%) as malignant. In multivariate analysis of clinical characteristics, large sized tumor (>20 mm, p=0.001) was confirmed as sole independent factor to predict malignant NET. Surgical resection was performed in 114 patients. All patients with benign NET are still alive without recurrence. Six out of 32 patients with malignant NET died at an average 40.3 months after diagnosis. The factors indicating favorable outcome were small size of tumors (p=0.046), resection of primary tumor (p=0.000), absence of lymph node invasion (p=0.0116) and distant metastasis (p=0.0005). CONCLUSIONS: Large NET of the pancreas, regardless of their functioning status, were more likely to be associated with malignancy and predictor of worse survival.
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Neuroendocrine Tumors/*diagnosis/mortality/pathology ; Pancreatectomy ; Pancreatic Neoplasms/*diagnosis/mortality/pathology ; Predictive Value of Tests ; Prognosis ; Retrospective Studies ; Survival Analysis

PURPOSE: The aim of this study is to report on preliminary data regarding the prevalence of major eye diseases in Korea. METHODS: We obtained data from the Korea National Health and Nutrition Examination Survey, a nation-wide cross-sectional survey and examinations of the non-institutionalized civilian population in South Korea (n = 14,606), conducted from July 2008 to December 2009. Field survey teams included an ophthalmologist, nurses, and interviewers, traveled with a mobile examination unit and performed interviews and ophthalmologic examinations. RESULTS: The prevalence of visual impairment, myopia, hyperopia and astigmatism in participants over 5 years of age was 0.4 +/- 0.1%, 53.7 +/- 0.6%, 10.7 +/- 0.4%, and 58.0 +/- 0.6%, respectively. The prevalence of strabismus and blepharoptosis in participants over 3 years of age was 1.5 +/- 0.1% and 11.0 +/- 0.8%, respectively. In participants over 40 years of age, the prevalence of cataract, pterygium, early and late age-related macular degeneration, diabetic retinopathy and glaucoma was 40.2 +/- 1.3%, 8.9 +/- 0.5%, 5.1 +/- 0.3%, 0.5 +/- 0.1%, 13.4 +/- 1.5%, and 2.1 +/- 0.2%, respectively. CONCLUSIONS: This is the first nation-wide epidemiologic study conducted in South Korea for assessment of the prevalence of eye diseases by both the Korean Ophthalmologic Society and the Korea Center for Disease Control and Prevention. This study will provide preliminary information for use in further investigation, prevention, and management of eye diseases in Korea.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Cross-Sectional Studies ; Eye Diseases/*epidemiology ; Female ; Humans ; Male ; Middle Aged ; Nutrition Surveys/*statistics & numerical data ; Prevalence ; Republic of Korea/epidemiology ; Young Adult