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.

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

The present study was to evaluate effects of vitamin E on intravenous administration of lidocaine-induced antinociception. Experiments were carried out using male Sprague-Dawley rats. Orofacial formalin-induced nociceptive behavioral responses were used as the orofacial animal pain model. Subcutaneous injection of formalin produced significant nociceptive scratching behavior. Intraperitoneal injection of 5 and 10 mg/kg of lidocaine attenuated formalin-induced nociceptive behavior in the 2nd phase, compared to the vehicle-treated group. Intraperitoneal injection of 1 g/kg of vitamin E also attenuated the formalin-induced nociceptive behavior in the 2nd phase, compared to the vehicle-treated group. However, low dose of vitamin E (0.5 g/kg) did not affect the nociceptive behavioral responses produced by subcutaneous injection of formalin. The present study also investigated effects of intraperitoneal injection of both vitamin E and lidocaine on orofacial formalin-induced behavioral responses. Vehicle treatment affected neither formalin-induced behavioral responses nor lidocaine-induced antinociceptive effects. However, intraperitoneal injection of 0.5 g/kg of vitamin E enhanced the lidocaine-induced antinociceptive effects in the 2nd phase compared to the vehicle-treated group. Intraperitoneal injection of naloxone, an opioid receptor antagonist, did not affect antinociception produced by intraperitoneal injections of both vitamin E and lidocaine. These results suggest that treatment with vitamin E enhances the systemic treatment with lidocaine-induced antinociception and reduces side effects when systemically treated with lidocaine. Therefore, the combined treatment with vitamin E and lidocaine is a potential therapeutic for chronic orofacial pain.
Administration, Intravenous ; Animals ; Facial Pain ; Formaldehyde ; Humans ; Injections, Intraperitoneal ; Injections, Subcutaneous ; Lidocaine* ; Male ; Naloxone ; Rats* ; Rats, Sprague-Dawley ; Receptors, Opioid ; Vitamin E* ; Vitamins*

Apert Syndrome is a kind of developmental disorder characterized by the craniosynostosis by synostosis of the coronal suture, bilateral symmetric syndactyly of the limbs (mitten-like hands and feet), midfacial hypoplasia, and variable degree of mental retardation. In 1894, Wheaton did the first description, and in 1906, it was named by Apert. Apert Syndrome is a rare autosomal dominent disorder and the prevalance at birth is estimated from 1:100000 to 160000. This syndrome is developed by the result of a mutation in the fibroblast growth factor receptor 2 gene (FGFR 2) located at 10q25.3-26. In familial cases, diagnosis in the first trimester sometimes has been made. But In sporadic cases, mostly it has been diagnosed in the second or third trimester by ultrasonography. In Korea, Apert syndrome is so rare, and it has not yet been reported that Apert syndrome is defined by prenatal molecular diagnosis with ultrasonographic detection. We present a case of prenatal molecular definitive diagnosis of Apert syndrome suspected strongly by ultrasonographic finding with a brief review of literature. Mother of affected fetus was transferred to our hospital at 31(2) weeks' gestation due to abnormal fetal ultrasound finding of severe polyhydroamnios and bilateral syndactyly of hands detected at 26(3) weeks' gestation. We suspected Apert syndrome by fetal ultrasonographic finding, and then confirmed Apert syndrome by DNA analysis of fetal amniocyte from therapeutic amnioredution at 31(4) weeks' gestation.
Acrocephalosyndactylia* ; Craniosynostoses ; Diagnosis* ; DNA ; Extremities ; Female ; Fetus ; Hand ; Humans ; Intellectual Disability ; Korea ; Mothers ; Parturition ; Pregnancy ; Pregnancy Trimester, First ; Pregnancy Trimester, Third ; Receptor, Fibroblast Growth Factor, Type 2 ; Sutures ; Syndactyly ; Synostosis ; Ultrasonography

BACKGROUND: Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by motor and nonmotor symptoms such as cognitive impairment and neuropsychiatric symptoms. The aim of this study was to investigate the spectrum of neuropsychiatric symptoms in PD according to cognitive function. METHODS: One hundred twenty seven patients with PD were consecutively recruited. They had undergone an intensive interview with a neurologist and the Seoul Neuropsychological Screening Battery-dementia version, and were divided into three groups: 27 patients in PD with normal cognition (PDNC), 57 in PD with mild cognitive impairment (PDMCI), and 43 in PD with Dementia (PDD). Forty five healthy controls without memory complaints were also recruited. The caregivers of all participants administered the Caregiver-Administered Neuropsychiatric Inventory (CGA-NPI). RESULTS: There were significant differences in the CGA-NPI score (22.8+/-20.9 vs. 6.4+/-10.1 vs. 1.7+/-3.9 vs. 1.0+/-1.6, p<0.001), and the prevalence of neuropsychiatric symptoms (100% vs. 64.9% vs. 37.0% vs. 44.4%, p<0.001) among PDD, PDMCI, PDNC and control groups. Depression was the most common symptom (43.3%), followed by anxiety (31.5%), apathy (26.8%), and night-time behavior (26.8%) in all PD patients. Delusion, hallucination, and aberrant motor behavior were observed frequently in PDD, but were rare in PDMCI. CONCLUSIONS: Depression was the most common neuropsychiatric symptom in PD. The presence of delusion, hallucination, or aberrant motor behavior may suggest PDD. The neuropsychiatric symptoms were not prevalent in PD with normal cognition.
Anxiety ; Apathy ; Caregivers ; Cognition ; Delusions ; Dementia ; Depression ; Hallucinations ; Humans ; Mass Screening ; Memory ; Mild Cognitive Impairment ; Neurodegenerative Diseases ; Parkinson Disease ; Prevalence

Primary thymic adenocarcinoma is a very rare malignancy of the anterior mediastinum with no standardized treatment. A 36-year-old male patient presented with hoarseness over the past 3 months. A chest computed tomography (CT) scan showed an infiltrative mass to the proximal vessels and aortic arch in left upper mediastinum (4.1x3.1x5.4 cm). Brain magnetic resonance imaging (MRI) showed focal lesions, suggesting metastasis in the left frontal lobe. A thoracoscopic biopsy of the mediastinal mass confirmed a primary thymic adenocarcinoma forming a glandular structure with atypia of tumor cells. The patient received four cycles of systemic chemotherapy, consisting of etoposide and cisplatin, with concurrent radiotherapy (6,000 cGy/30 fractions) to the mediastinal lesion and the metastatic brain lesion (4,200 cGy/12 fractions). A follow-up chest CT scan and brain MRI showed a decrease in the size of the left upper mediastinal mass and brain lesion. We report a rare case of the primary thymic adenocarcinoma with a literature review.
Adenocarcinoma* ; Adult ; Aorta, Thoracic ; Biopsy ; Brain ; Chemoradiotherapy ; Cisplatin ; Drug Therapy ; Etoposide ; Follow-Up Studies ; Frontal Lobe ; Hoarseness ; Humans ; Magnetic Resonance Imaging ; Male ; Mediastinum ; Neoplasm Metastasis ; Radiotherapy ; Small Cell Lung Carcinoma* ; Thorax ; Thymus Gland ; Tomography, X-Ray Computed