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

Primary carcinoma of the fallopian tube is an extremely rare malignancy of the female genital tract. We have experienced two cases of primary carcinoma in the fallopian tube. One case with huge mass which was diagnosed incidentally on the basis of the biopsy findings, and the other case of the 3 years follow-up. We reported the cases with a brief review of the concemed literatures.
Biopsy ; Fallopian Tubes* ; Female ; Follow-Up Studies ; Humans

Benign cystic teratoma is the common ovarian tumor, and the incidence reported about 20%. Malignant transformation of mature cystic teratoma of the ovary is rare, which can develop with an incidence of less than 2%. The most common malignant tumor arising in mature cystic teratoma is squamous cell carcinoma which account for 75-85%, but vary rare cases of anaplastic carcinoma arising from mature cystic teratoma has been reported all over the world and prognosis of this malignant tumor was poor. No case of anaplastic carcinoma has been reported before in Korea. We experienced a case of anaplastic carcinoma arising from mature cystic teratoma of the ovary and report our case with a brief review of literature.
Carcinoma* ; Carcinoma, Squamous Cell ; Female ; Incidence ; Korea ; Ovary* ; Prognosis ; Teratoma*

Background: To evaluate how intrauterine stress affects extremely premature infants in terms of intrauterine growth restriction. We hypothesized that extremely premature infants with mildly-low ponderal index (MPI) would have better neonatal outcomes. Methods: We selected 2,721 subjects of 23 to 28 weeks of gestation between 2013 and 2015 from Korean Neonatal Network database. They were divided into 4 groups based on ponderal index (PI) percentile; PI ≤ 3rd as severely-low PI (SPI, n = 82), 3rd < PI ≤ 10th as MPI (n = 190), 10th < PI ≤ 90th as adequate PI (API, n = 2,179), and PI > 90th as high PI (HPI, n = 270). Results: The mortality in MPI and API groups was comparable (16.3% vs. 16.9%). It was significantly lower than that in the SPI and HPI groups (30.5% and 24.9%, respectively;P = 0.001). The MPI and API groups had better neonatal morbidities compared with the SPI and/or HPI groups, while the MPI group (8.2%) showed a lower incidence of severe intraventricular hemorrhage (IVH) than the other groups (SPI, 21.3%; API, 15.0%; HPI, 19.7%, respectively; P = 0.004). The MPI group had a trend of a bottom in neonatal mortality and morbidities in extremely premature infants. Conclusion The MPI and API groups had lower mortality, massive pulmonary hemorrhage, severe bronchopulmonary dysplasia or death, pulmonary hypertension and neonatal seizure rates than the SPI and/or HPI groups, while the MPI group showed a lower incidence of severe IVH than the other groups. We speculate that the lower incidence of neonatal morbidities and mortality in the MPI group indicating mild intrauterine stress might accelerate fetal maturation resulting in better outcomes in extremely premature infants.

Pericardial effusion (PCE) in neonates has various clinical presentations depending on the amount and speed of fluid accumulation and can cause cardiac tamponade (CT). We report a case of rapidly accumulating PCE and near-fatal CT with an umbilical venous catheter successfully resolved by emergent echo-guided pericardiocentesis in a term infant who had been hospitalized with meconium aspiration syndrome and persistent pulmonary hypertension. This case report suggests that if a patient with an intracardiac umbilical catheter shows sudden cardiopulmonary instability, the possibility of PCE and CT should be considered. Furthermore, if necessary, emergency drainage of the PCE and removal of the umbilical catheter should be immediately performed.

Objective: This study aimed to evaluate the performance of an integrated risk stratification system (RSS) based on ultrasound (US) RSSs, nodule size, and cytology subcategory for diagnosing malignancy in thyroid nodules initially identified as Bethesda category III on fine-needle aspiration. Materials and Methods: This retrospective study was conducted at two institutions and included consecutive patients with Bethesda category III nodules, and final diagnoses confirmed by repeat biopsy or surgery. A total of 320 Bethesda category III nodules (≥1 cm) from 309 patients (223 female and 86 male; mean age, 50.9 ± 12.0 years) were included. The malignancy risk of Bethesda category III nodules and predictors of malignancy were assessed according to US RSSs, nodule size, and cytology subcategory. The diagnostic performances of US-size cytology (USC) RSS and US RSS alone for malignancy were compared. Results: The intermediate or high suspicion US category independently increased the malignancy risk in all US RSSs (P ≤ 0.001). Large nodule size (≥3 cm) independently increased the malignancy risk of low- or intermediate suspicion US category nodules. Additionally, the atypia of undetermined significance cytology subcategory independently increased the malignancy risk of low suspicion US category nodules in most US RSSs. The area under the receiver operating characteristic curve of the USC RSSs was greater than that of the US RSSs alone (P < 0.048). Malignancy was not found in the very low risk category of USC RSS. Conclusion The diagnostic performance of USC RSS for malignancy was superior to that of US RSS alone in Bethesda category III nodules. Malignancy can be ruled out in the very low-risk category of USC RSS.

Our society is aging rapidly, and the number of elderly people who are in charge of purchasing and preparing foods at home is increasing. However, most the elderly have difficulty managing nutrition and food safety by themselves. The purpose of this study was to develop the necessary knowledge, through focus group interview and Delphi technique to establish a food safety and nutrition education program. The diet and educational needs of the elderly were surveyed through FGI. The education topics were decided by the Delphi technique. The education program consisted of a five week program, and the topics taught were 'Dietary Change for Healthy Life', 'Prevention of Food Poisoning in Everyday Life', 'Safe Food Handling for my Health', 'Healthy Dietary Life to Prevent Chronic Disease', and 'Safety! Nutrition! Healthy Dietary Life'. This education program was designed to decrease the perceived barriers, and to increase the perceived interests and the sense of self-efficacy. Education program materials, lesson plans, slides, handouts, videos, leaflets, and booklets were developed. Based on the results, the contents of the food safety guideline leaflets for the elderly were decided as the following: (1) wash your hands in the correct way; (2) select safe food; (3) cook foods safely; (4) keep foods safely; (5) keep kitchen utensils clean. In conclusion, if advanced education programs are implemented and delivered continuously in locations such as health centers and community welfare centers, those will contribute significantly to enhance the perception of food safety and to change the desirable dietary behavior of the elderly.
Aged ; Aging ; Delphi Technique ; Diet ; Fees and Charges ; Focus Groups ; Food Handling ; Food Safety ; Foodborne Diseases ; Hand ; Humans ; Pamphlets

PURPOSE: Kawasaki disease (KD) is the main cause of acquired heart disease in children. In addition to cardiovascular involvement, many complications have been recognized in KD. However, respiratory complications have been rarely reported. We investigated the differences in clinical characteristics, laboratory findings, radiography findings, and echocardiography findings of Mycoplasma pneumoniae infection and other types of pneumonia in KD patients. METHODS: Among 358 patients with KD, 54 developed concurrent pneumonia. Among the 54 patients, 12 (22.2%) with high titers of anti-M. pneumoniae antibody (AMA) (>1:640) were grouped in the M. pneumoniae group and 42 were included in the control group. Serum AMA was measured in each patient. Clinical laboratory findings and total duration of fever were analyzed. RESULTS: The duration of fever, serum hemoglobin, white blood cell count, platelet count, erythrocyte sedimentation rate, C-reactive protein level, albumin level, and the incidence of coronary arterial lesions showed no statistical difference in the 2 groups. Neutrophil count was significantly higher in the M. pneumoniae group than in the control group. Among various radiography findings observed in pneumonia, consolidation and pleural effusion were more frequent in the M. pneumoniae group than in the control group. On the other hand, parahilar peribronchial opacification, diffuse interstitial lesion, and normal findings prevailed in the control group. CONCLUSION: KD patients can have concurrent infections, especially pulmonary symptoms. The cause of KD is likely to be associated with M. pneumoniae infection. Thus, immediate treatment of M. pneumoniae infection in KD patients is very important.
Blood Sedimentation ; C-Reactive Protein ; Child ; Echocardiography ; Fever ; Hand ; Heart Diseases ; Hemoglobins ; Humans ; Incidence ; Leukocyte Count ; Mucocutaneous Lymph Node Syndrome ; Mycoplasma ; Mycoplasma pneumoniae ; Neutrophils ; Platelet Count ; Pleural Effusion ; Pneumonia ; Pneumonia, Mycoplasma

BACKGROUND: Cerebral small vessel disease (SVD) induces vascular cognitive impairment (VCI) such as subcortical vascular dementia (SVaD) and subcortical vascular mild cognitive impairment (svMCI). We compared MRI parameters between SVaD and svMCI and determined which MRI parameters best correlated with cognitive function and disability on cross-sectional and longitudinal analyses within them. METHODS: Twenty-four patients with SVaD and twelve with svMCI were recruited. They underwent multimodal MRIs including fluid-attenuated inversion recovery lesion load, lacunar infarct number, and fractional anisotropy (FA) and mean diffusivity (MD) from diffusion tensor imaging (DTI), neuropsychological testing, Sum of Boxes of Clinical Dementia Rating Scale (CDR-SB), Barthel Index, and the Korean version of a Geriatric Depression Scale (GDS-K). Seventeen patients were retested after 20 months for a brain MRI and clinical evaluation. RESULTS: There were significant differences in average MD and peak height of MD histograms within normal-appearing brain tissue (NABT) between SVaD and svMCI patients. In the cross-sectional analysis, average MD within NABT significantly correlated with the composite neuropsychology score (r=-0.80, p<0.001), the composite executive function score (r=-0.67, p< 0.001), and the CDR-SB (r=0.54, p=0.001), and the Barthel Index correlated with peak heights of the MD histograms (r=0.37, p=0.03) in NABT. Changes of CDR-SB was associated with changes of average MD within WMH (r=0.57, p=0.02), and changes of GDS-K was associated with changes of WMH volume (r=0.51, p=0.04) on a longitudinal scale. CONCLUSIONS: DTI parameters in NABT correlated with cognitive impairment and disability in VCI associated with SVD. Clinical progression of SVD was associated with some increment of WML volume and ultrastructural changes in WMH.
Anisotropy ; Brain ; Cerebral Small Vessel Diseases ; Cross-Sectional Studies ; Dementia ; Dementia, Vascular ; Depression ; Diffusion ; Diffusion Tensor Imaging ; Executive Function ; Glycosaminoglycans ; Humans ; Mild Cognitive Impairment ; Neuropsychological Tests ; Neuropsychology ; Stroke, Lacunar

Larrea nitida is a plant that belongs to the Zygophyllaceae family and is widely used in South America to treat inflammatory diseases, tumors and menstrual pain. However, its pharmacological activity remains unclear. In this study we evaluated the property of selective estrogen receptor modulator (SERM) of Larrea nitida extracts (LNE) as a phytoestrogen that can mimic, modulate or disrupt the actions of endogenous estrogens, depending on the tissue and relative amount of other SERMs. To investigate the property of SERM of LNE, we performed MCF-7 cell proliferation assays, estrogen response element (ERE)-luciferase reporter gene assay, human estrogen receptor (hER) binding assays and in vivo uterotrophic assay. To gain insight into the active principles, we performed a bioassay-guided analysis of LNE employing solvents of various polarities and using classical column chromatography, which yielded 16 fractions (LNs). LNE showed high binding affinities for hERalpha and hERbeta with IC50 values of 1.20x10(-7) g/ml and 1.00x10(-7) g/ml, respectively. LNE induced 17beta-estradiol (E2)-induced MCF-7 cell proliferation, however, it reduced the proliferation in the presence of E2. Furthermore, LNE had an atrophic effect in the uterus of immature rats through reducing the expression level of progesterone receptor (PR) proteins. LN08 and LN10 had more potent affinities for binding on hER alpha and beta than other fractions. Our results indicate that LNE had higher binding affinities for hERbeta than hERalpha, and showed SERM properties in MCF-7 breast cancer cells and the rat uterus. LNE may be useful for the treatment of estrogen-related conditions, such as female cancers and menopause.
Animals ; Breast Neoplasms ; Chromatography ; Dysmenorrhea ; Estrogens* ; Female ; Genes, Reporter ; Humans ; Inhibitory Concentration 50 ; Larrea* ; MCF-7 Cells* ; Menopause ; Phytoestrogens ; Plants ; Rats* ; Receptors, Progesterone ; Response Elements ; Selective Estrogen Receptor Modulators ; Solvents ; South America ; Uterus* ; Zygophyllaceae