Leigh's disease is a rare progressive neurological disorder that is characterized light microscopically by focal spongy necrosis in the brain and electron microscopically by mitochondriopathy. We report an autopsy case of Leigh's disease that showed abnormalities in the liver, kidney and skeletal muscle as well as the central nervous system. The patient was an 18-month-old girl who has carried a diagnosis of cerebral palsy ever since her birth to a 20-year-old mother. The baby was generally hypertonic and mentally retarded. She died of severe metabolic acidosis. Postmortem examination showed growth retardation, fatty liver, fatty kidney and soft brain. Brain section showed multifocal softenings in the brainstem, basal ganglia and periventricular areas. Microscopically increased capillaries with endothelial proliferation, vacuolar degeneration and mild gliosis were seen in the brain. The axons were relatively preserved. Liver and kidneys showed microvesicular fatty change. Myofiber degeneration of the skeletal muscle was also noted. Electron microscopic examination showed markedly increased mitochondria in the parenchymal cells of the brain, liver and kidney. The mitochondria showed round to ovoid ballooned appearance including electron-dense core-like structures and pseudoinclusions of glycogen granules.
Brain/pathology/ultrastructure ; Female ; Humans ; Infant ; Kidney/pathology/ultrastructure ; Leigh Disease/*pathology ; Liver/pathology/ultrastructure ; Mitochondrial Encephalomyopathies/pathology ; Muscles/pathology

Twin conceptus of a complete hydatidiform mole (CHM) and a normal fetus are rare but are important because of diagnostic difficulty, problems related to twin pregnancy, and high risk of persistent gestational trophoblastic tumor. Recently, we experienced one case of twin pregnancy consisting of a CHM and a normal fetus. A 26-year-old woman complained of vaginal bleeding. She had evidences of pregnancy-induced hypertension. A male fetus was delivered at 20 gestational weeks. The placenta demonstrated vesicles of molar change separated from normal placenta. Microscopically, the molar villi disclosed diffuse hydropic swelling with circumferential trophoblastic proliferation. DNA flow cytometric analysis showed diploid patterns in both molar and normal placental tissues. Fluorescent in situ hybridization in paraffin-embedded tissue presented that normal placental villi hybridized with X- and Y-chromosome probes (46, XY), while molar villi hybridized with X-chromosome only (46, XX). Thus, dizygotic twinning was confirmed because sex differences were shown between molar villi and normal placental villi. Follow up beta-hCG was within normal range after delivery.
Adult ; Chorionic Villi ; Diploidy ; DNA ; Female ; Fetus* ; Follow-Up Studies ; Humans ; Hydatidiform Mole* ; Hypertension, Pregnancy-Induced ; In Situ Hybridization, Fluorescence ; Male ; Molar ; Placenta ; Pregnancy ; Pregnancy, Twin ; Reference Values ; Sex Characteristics ; Trophoblastic Neoplasms ; Trophoblasts ; Twins ; Twins, Dizygotic ; Uterine Hemorrhage

PURPOSE: To investigate the levels of uncertainty and anxiety at admission and discharge to the isolation unit for patients undergoing hematopoietic stem cell transplantation (HSCT). METHOD: The data were collected from 60 patients who were admitted to the department of HSCT. Uncertainty and anxiety were assessed using the Mishel Uncertainty in Illness Scale (MUIS) and Anxiety Inventory (STAI). Collected data were analyzed using the SAS program. RESULTS: The mean score for anxiety at admission was 2.20, and at discharge 2.10. The mean score for uncertainty at admission was 2.19, and at discharge 2.07. The anxiety at admission the group with physical discomfort was higher than that of group without physical discomfort. A positive relationship was found between anxiety and uncertainty at admission and at discharge. The major variables were expectation for cure and physical discomfort, explaining 25.87% of the anxiety at admission. The major variable was expectation for cure, explaining 20.94% of the uncertainty at admission. CONCLUSION: From the above results, it can be concluded that support and consideration are required to reduce anxiety of the inpatient in single room.
Anxiety* ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Inpatients ; Stem Cell Transplantation ; Uncertainty*

BACKGROUND/OBJECTIVES: The purpose of this study was to develop a sodium index, which is a tool for estimating and assessing sodium intake easily and quickly, to assist in the prevention of various diseases induced by excess sodium intake in Korean adults. SUBJECTS/METHODS: The 24-h urine collection and dietary behavior surveys were performed on 640 healthy people in 4 regions of South Korea, and an equation for the estimation of 24-h sodium intake was developed. The validity and reliability of the equation were verified with 200 adults. The sodium index was developed by converting the estimated sodium intake using the equation. Finally, the sodium intake status of 1,600 adults was assessed using the sodium index. RESULTS: The equation included sex, age, body mass index, eating habit and dietary behaviors related to sodium intake. In validity test of the equation, the mean bias between sodium intake using 24-h urine analysis and using the equation from the Bland-Altman plots was −1.5 mg/day. The sensitivity and specificity of the equation for estimation of sodium intake were 80.5% and 64.4%, respectively. In the reliability test of the equation, there was no significant difference between the first and second sodium intakes calculated using the equations, and Spearman's correlation coefficient between the 2 sodium intakes was 0.98. Sodium intake can be assessed as ‘very moderate’ for 75–100 on the sodium index, ‘moderate’ for 100–150, ‘careful’ for less than 75 or 150–200, and ‘severe’ for 250 or more. When sodium intake was assessed using the sodium index in 1,600 subjects, 54.3% and 24.3% of the subjects were assessed to be in the ‘careful’ and ‘severe’ categories, respectively. CONCLUSIONS Using a simple questionnaire, the sodium index can be used to monitor and assess sodium intake status, assisting in nutrition education and counseling in a large population.

The purpose of this study was to investigate the association between daily beverage intake and obesity in young adults. This cross-sectional study was conducted with 256 adults in their 20s and 30s. Obesity was evaluated by anthropometric measurement of body mass index (BMI), waist to hip ratio and waist to height ratio. In addition, the daily intake of milk, caffeinated beverages, sweetened beverages, and alcoholic beverages was investigated using the dietary record and 24-h recall methods. Based on the BMI, men and women were classified into underweight, normal, overweight and obese. Among men, 38.3% were found to be normal, 28.1% were overweight and 31.3% obese. Among women, 11.7% were underweight, 73.4% were normal, and 10.2% were overweight, which was significantly different from men. In women, the BMI of the milk consumers was significantly lower than the non-consumers. Also, the obesity indices of caffeinated beverage consumers were significantly lower than the non-consumers. An analysis of the correlation between beverage intake and the obesity index showed that the intake of caffeinated beverages among women had a significant negative correlation with the obesity index, and intake of alcoholic beverages had a significant positive correlation with waist to height ratio. This study suggests that intake of milk and caffeinated beverage may have a positive effect on obesity, whereas the intake of alcoholic beverages may have a negative effect, especially in young adult women.

BACKGROUND/OBJECTIVES: This study aimed to compare 24-h diet recall (DR) and 24-h urine collection (UC) for estimating sodium and potassium intakes and their ratio (Na/K), identifying factors associated with sodium and potassium intakes and Na/K, and identifying those who were likely to underestimate sodium and potassium intakes by DR. SUBJECTS/METHODS: A total of 640 healthy adults aged 19–69 yrs completed a questionnaire survey, salty taste assessment, anthropometric measurement, two 24-h DRs, and two 24-h UCs. RESULTS: The mean sodium and potassium intakes and Na/K were 3,755 mg/d, 2,737 mg/d, and 1.45 according to DR, and 4,145 mg/d, 2,812 mg/d, and 1.57 according to UC, with percentage differences of −9.4%, −2.7%, and −7.6% in the values between the two methods, respectively.Men, older adults, smokers, obese individuals, those who consumed all the liquid in the soup, and those who were found to be salty in the salty taste assessment consumed significantly more sodium; older adults, the heavy- activity group, and obese individuals consumed more potassium; and men, younger adults, smokers, and obese individuals had a significantly higher Na/K, according to UC. Compared with UC, DR was more likely to underestimate sodium intake in older adults, smokers, obese individuals, those who consumed all the liquid in the soup, and those who consumed eating-out/delivery food at least once a day, and potassium intake in older adults, the heavy-activity group, and obese individuals. CONCLUSIONS The mean sodium and potassium intakes and Na/K estimated by DR were comparable to those measured by UC. However, the association of sodium and potassium intakes with sociodemographic and health-related factors showed inconsistent results when estimated by DR and UC. Factors influencing the underestimation of sodium intake by DR compared to UC should be further investigated.

Background: Chronic glucocorticoid exposure is associated with resistance to nondepolarizing neuromuscular blocking agents. Therefore, we hypothesized that sugammadex-induced recovery would occur more rapidly in subjects exposed to chronic dexamethasone compared to those who were not exposed. This study evaluated the sugammadex-induced recovery profile after neuromuscular blockade (NMB) in rats exposed to chronic dexamethasone. Methods: Sprague–Dawley rats were allocated to three groups (dexamethasone, control, and pair-fed group) for the in vivo study. The mice received daily intraperitoneal dexamethasone injections (500 μg/kg) or 0.9% saline for 15 days. To achieve complete NMB, 3.5 mg/kg rocuronium was administered on the sixteenth day. The recovery time to a train-of-four ratio ≥ 0.9 was measured to evaluate the complete recovery following the sugammadex injection. Results: Among the groups, no significant differences were observed in the recovery time to a train-of-four ratio ≥ 0.9 following sugammadex administration (P = 0.531). The time to the second twitch of the train-of-four recovery following rocuronium administration indicated that the duration of NMB was significantly shorter in Group D than that in Groups C and P (P = 0.001). Conclusions Chronic exposure to dexamethasone did not shorten the recovery time of sugammadex-induced NMB reversal. However, the findings of this study indicated that no adjustments to sugammadex dosage or route of administration is required, even in patients undergoing long-term steroid treatment.

Behcet's disease (BD) is a chronic relapsing multisystem disease characterized by oral ulceration, genital ulceration and ocular lesions. Gastrointestinal involvement is rare, often difficult to treat and associated with a high mortality rate. We treated a 47-year-old Korean man with BD who had a recurrent intestinal ulcer with tumor necrosis factor alpha antibody (infliximab); he initially underwent right hemicolectomy due to uncontrolled intestinal bleeding. For patients with intestinal BD who fail to respond to conventional treatment, infliximab may be a safe and effective new therapeutic option.
Tumor Necrosis Factor-alpha/*therapeutic use ; *Treatment Outcome ; Remission Induction ; Middle Aged ; Male ; Humans ; Gastrointestinal Diseases/*drug therapy/etiology/surgery ; Gastrointestinal Agents/*therapeutic use ; Disease Progression ; Colectomy ; Behcet Syndrome/*drug therapy/physiopathology/surgery ; Antibodies, Monoclonal/*therapeutic use

Four cases of primary transitional cell carcinoma (TCC) arising in the ovary (3 cases) and the parovarium (1 case) were collected for clinicopathologic analysis. The mean age was 46.2 years (range, 39-57 years). Two patients complained abdominal discomfort and vaginal discharge, respectively. Other 2 cases were incidentally found from routine check. Grossly, the tumors were solid and cystic (2 cases), solid (1 case) and surface papillary growth on capsule (1 case). Microscopically, the tumor showed almostly same to the histologic features of TCC of urinary bladder. Three cases were pure TCC, and one was mixed TCC and serous carcinoma. FIGO stage were 1 IIa, 2 IIc, and 1 IIIc. Treatment was surgery with adjuvant chemotherapy. Two patients are alive with no evidence of disease, and two have lung or brain metastasis.
Brain ; Carcinoma, Transitional Cell* ; Chemotherapy, Adjuvant ; Female ; Humans ; Lung ; Neoplasm Metastasis ; Ovary ; Urinary Bladder ; Vaginal Discharge

Although it has been proposed that trace minerals have anti-oxidative functions and are related to the control of blood pressure, only a limited number of studies directly address the issue. Thus, the purpose of our study was to assess the intake of copper and manganese, which are trace minerals, and to clarify their relation to blood pressure. In a cross-sectional study, the blood pressure of 640 normotensive adults, from 19 to 69 year-old (320 males and 320 females), was measured, and its correlation with the intake of copper and manganese was assessed using a 24-hour dietary recall method. The average value of the blood pressure was 126.4/80.2 mmHg for the males and 117.8/75.8 mmHg for the females. The daily copper intake was 1.3 mg/day for the males and 1.2 mg/day for the females. For manganese, the daily intake was 4.2 mg/day for the males and 4.1 mg/day for the females. Although the copper intake of all subjects showed a positive correlation with the systolic and diastolic blood pressures, there was no significant correlation when the potential confounding factors were adjusted. The manganese intake of the male subjects had a significantly negative correlation with the systolic blood pressure after adjusting for gender, age, body mass index, and energy intake. In conclusion, the daily manganese intake of the normotensitve adults showed a significantly negative correlation with the systolic blood pressure indicating a possibility of a positive effect of manganese on blood pressure.
Adult* ; Blood Pressure* ; Body Mass Index ; Copper* ; Cross-Sectional Studies ; Energy Intake ; Female ; Humans ; Male ; Manganese* ; Minerals