Behcet's syndrome has been known as a disease which has the triple symptoms such as recurrent orogenital ulceration and eye inflammation. The syndrome is now recogninized as a multisystem disease involving mucocutaneaous, cardiovascular, central nervous system, renal, articular and gastrointestinal system. The vascular involvement in Behcet's disease has been reported since Mischima first described the case in 1961, four types of vascular lesion are recognized ; arterial occlusion ; aneurysm ; venous occlusion ; and varices. Venous occlusion are frequently observed, most commonly in the inferior or superior vena cava. We report a case of Behcet's disease manifested as superior vena cava syndrome due to thrombus on both brachiocephalic veins.
Aneurysm ; Behcet Syndrome* ; Brachiocephalic Veins ; Central Nervous System ; Inflammation ; Superior Vena Cava Syndrome* ; Thrombosis ; Ulcer ; Varicose Veins ; Vena Cava, Superior*

We report here on two cases of testicular torsion in the inguinal canal, and there have been only rare published reports on this condition. Doppler ultrasound was used for making the diagnosis in the present two cases, which were initially considered to be inguinal hernia.
Child ; Cryptorchidism ; Hernia, Inguinal ; Humans ; Inguinal Canal ; Male ; Spermatic Cord Torsion

No abstract available.
Brachial Plexus ; Sternotomy

Right intrathoracic stomach associated with organoaxial torsion is a rare form of congenital hiatal hernia. We report the radiologic findings in two cases of complete or partial right intrathoracic stomach secondary to congenital hiatal hernia. The barium meal test demonstrated the presence of complete or partial right intrathoracic stomach and non-obstructive organoaxial torsion with the greater curvature lying against the right chest wall. The esophagogastric junction was located above the diaphragm. CT revealed a cystic mass in the right posterior mediastinum. This cystic lesion should be differentiated from other congenital mediastinal cysts.
Barium ; Deception ; Diaphragm ; Esophagogastric Junction ; Hernia, Hiatal* ; Meals ; Mediastinal Cyst ; Mediastinum ; Stomach* ; Thoracic Wall

PURPOSE: The renin-angiotensin system (RAS) has been demonstrated to play a major role in regulating blood pressure. Therefore, components of the RAS are likely candidate genes that may predispose an individual to essential hypertension and cardiovascular complications. Among them, the M235T polymorphism of the angiotensinogen gene has been speculated to be associated with elevated circulating angiotensinogen concentrations and essential hypertension. This study aimed to analyze the angiotensinogen M235T polymorphism in hypertensive adolescents and investigate its relationship with cardiovascular risks. METHODS: Forty Korean hypertensive adolescents (aged 16-17, systolic BP > or =140 mmHg and/or diastolic BP > or =90 mmHg) and fifty seven normal adolescents were included. Obesity index (OI), body mass index (BMI) were calculated. BP was measured by oscillometric methods in resting state. Polymerase chain reaction (PCR) technique was performed on DNA from the hypertensives subjects to analyze the M235T polymorphism. Serum homocysteine, insulin, renin, aldosterone and angiotensin converting enzyme (ACE) were tested according to each genotype. The carotid intima-media thickness (IMT) and carotid artery diameter, Pulse wave velocity (PWV) and ankle-brachial index (ABI) were measured according to each genotype. RESULTS: Genotype frequencies of T/T, M/T and M/M were 62.5%, 35.0%, 2.5%, respectively in hypertensive adolescents. The results were not significantly different compared to control group. Serum insulin, renin levels, BMI and OI were significantly higher in thoses with the M/M genotype as compared to those with the T/T of M/T genotype. CONCLUSION: This study showed that the M235T polymorphism was not associated with essential hypertension or any cardiovascular risks. Further clinical research is required to ascertain the relationship between this polymorphism and cardiovascular complications in Korean hypertensive adolescents.
Adolescent ; Aldosterone ; Angiotensinogen ; Ankle Brachial Index ; Blood Pressure ; Body Mass Index ; Carotid Arteries ; Carotid Intima-Media Thickness ; DNA ; Genotype ; Homocysteine ; Humans ; Hypertension ; Insulin ; Obesity ; Peptidyl-Dipeptidase A ; Polymerase Chain Reaction ; Pulse Wave Analysis ; Renin ; Renin-Angiotensin System

Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor of uncertain origin, and it has a strong propensity for metastasis to the lungs, bones and brain. We report upon an unusual case of ASPS, presenting as multiple lung nodules with no other detectable primary site, in a 44-year-old man. A fine needle aspiration of the nodules yielded scattered, discohesive cells, each containing an eccentrically displaced nucleus and prominent nucleolus, on a granular background. Tumor cells with numerous bared nuclei, and occasional sheets of epithelioid cells were also found. Under the cytological diagnosis of an unclassified epithelioid malignant tumor, resection of the lung nodules was performed. The histologic findings were consistent with ASPS, showing positive TFE3-nuclear immunoreactivity. There is limited literature concerning cytological findings associated with pulmonary ASPS: especially in cases where the primary site is unknown. Here, we present a cytological review of pulmonary ASPS, investigating the significance of TFE3 staining in the diagnosis of ASPS.
Adult ; Biopsy, Fine-Needle ; Brain ; Epithelioid Cells ; Humans ; Lung ; Neoplasm Metastasis ; Sarcoma, Alveolar Soft Part ; Viperidae

Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma composed of dilated folliculosebaceous units and mesenchymal elements. It presents as a papule or nodule usually on the face and scalp, rarely on the genital or trunk area. Histologically, FSCH shares several similar features to sebaceous trichofolliculoma. We report one case of FSCH misdiagnosed as a neurofibroma. He was a 38-year-old man with a neurofibromatosis type I and a nodule on his left earlobe was excised under the impression of neurofibroma. Pathological examination revealed FSCH. Although FSCH is clinically not distinctive, awareness of the lesion is important to differentiate papulonodular or cyst-like cutaneous lesions.
Adult ; Follicular Cyst ; Hamartoma ; Humans ; Neoplasms, Basal Cell ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Scalp ; Skin Neoplasms

No abstract available.
Brain* ; Cerebral Infarction ; Hyperhomocysteinemia* ; Vitamin B 12 Deficiency* ; Vitamin B 12* ; Vitamins*

BACKGROUND: Oral isotretinoin is an effective treatment modality for acne, but this agent may cause dryness of the skin and mucous membrane. Gamma-linolenic acid (GLA) has been known to improve skin moisture and reduce transepidermal water loss (TEWL). It is also known to have anti-inflammatory effects and to inhibit 5-alpha reductase. OBJECTIVE: This study was performed to evaluate the clinical efficacy of GLA in the prevention of xerosis and improvement of acne lesions after concomitant administration of isotretinoin. METHODS: In this prospective study, 102 Korean acne patients were enrolled and randomized to receive isotretinoin either with or without GLA for 12 weeks. Comparing evaluations included skin hydration, TEWL, visual analogue scale (VAS) score for dryness of the lips and skin, number of acne lesions such as comedos, papules, and pustules, Korean acne grading system (KAGS) grade, and patient satisfaction regarding treatment. RESULTS: The decrease of skin hydration and increase of the TEWL of the lips and cheek were less definite in the experimental group than those of the control group. VAS score for dryness of the lips and skin was significantly lower in the experimental group compared with the control group. The number of acne lesions decreased in both groups; only the numbers of papules were different between the two groups. The decrease of the KAGS grade was more definite in the experimental group than that in the control group. CONCLUSION: This study suggests that GLA could be used effectively for the improvement of acne lesions as well as for the prevention of xerosis.
Acne Vulgaris* ; Cheek ; gamma-Linolenic Acid* ; Humans ; Isotretinoin* ; Lip ; Mucous Membrane ; Oxidoreductases ; Patient Satisfaction ; Prospective Studies ; Skin

Background: and Purpose: The expression of the 18-kDA mitochondrial translocator protein (TSPO) in the brain is an attractive target to study neuroinflammation. However, the binding properties of TSPO ligands are reportedly dependent on genetic polymorphism of the TSPO gene (rs6971). The objective of this study is to investigate the rs6971 gene polymorphism in the Korean population. Methods: We performed genetic testing on 109 subjects including patients with mild cognitive impairment, Alzheimer’s disease (AD) dementia, non-AD dementia, and cognitively unimpaired participants. Magnetic resonance imaging scans and detailed neuropsychological tests were also performed, and 29 participants underwent 18 F-DPA714 PET scans. Exon 4 of the TSPO gene containing the polymorphism rs6971 (Ala or Thr at position 147) was polymerase chain reaction amplified and sequenced using the Sanger method. The identified rs6971 genotype codes (C/C, C/T, or T/T) of the TSPO protein generated high-, mixed-, or lowaffinity binding phenotypes (HABs, MABs, and LABs), respectively. Results: We found that 96.3% of the study subjects were HAB (105 out of 109 subjects), and 3.7% of the subjects were MAB (4 out of 109 subjects). 18 F-DPA-714 PET scans showed nonspecific binding to the thalamus and brainstem, and increased tracer uptake throughout the cortex in cognitively impaired patients. The participant with the MAB polymorphism had a higher DPA714 signal throughout the cortex. Conclusions The majority of Koreans are HAB (approx. 96%). Therefore, the polymorphism of the rs6971 gene would have a smaller impact on the availability of second-generation TSPO PET tracers.