Homocystinuria is an inborn error of methionine metabolism and has several causes. Among the causes, cystathionine-b-synthase deficiency is the most common. The major clinical manifestations are ectopia lectis skeletal deformities, mental retardation and occlusive vascular disease A 16 year old girl was admitted with generalized seizure. She had a history of bilateral lens dislocation and thoracic scoliosis. Her brain MRI and MRA showed mass-like lesions at both frontal area and diffuses, stenosis of the right internal carotid artery She underwent a stereotaxic brain biopsy and cerebral angiography. Two days, after angiography, she was suddenly aggravated to show stuporous mentality and quadriplegia. FoIlow-up brain MRI showed newly developed acute ischemic lesions at both parietal area MR venography confirmed superior sagittal sinus thrombosis. Methionine and homocystine were markedly elevated in plasma and 24 hour urine. She recovered with anticoagulation and vitamin supplementation(folate and pyridoxine). Homocystinuria should be suspected in stroke patients of young age, especially if thy have nontraumatic lens dislocation or marfanoid features. We report a patient with homocystinuria complicated by cerebral venous sinus thrombosis which was aggrevated after cerebral angiography.
Adolescent ; Angiography ; Biopsy ; Brain ; Carotid Artery, Internal ; Cerebral Angiography ; Congenital Abnormalities ; Constriction, Pathologic ; Female ; Homocystine ; Homocystinuria* ; Humans ; Intellectual Disability ; Lens Subluxation ; Magnetic Resonance Imaging ; Metabolism ; Methionine ; Phlebography ; Plasma ; Quadriplegia ; Rabeprazole ; Scoliosis ; Seizures ; Sinus Thrombosis, Intracranial ; Stroke ; Stupor ; Superior Sagittal Sinus* ; Thrombosis* ; Vascular Diseases ; Vitamins

Homocystinuria is an inborn error of methionine metabolism and has several causes. Among the causes, cystathionine-b-synthase deficiency is the most common. The major clinical manifestations are ectopia lectis skeletal deformities, mental retardation and occlusive vascular disease A 16 year old girl was admitted with generalized seizure. She had a history of bilateral lens dislocation and thoracic scoliosis. Her brain MRI and MRA showed mass-like lesions at both frontal area and diffuses, stenosis of the right internal carotid artery She underwent a stereotaxic brain biopsy and cerebral angiography. Two days, after angiography, she was suddenly aggravated to show stuporous mentality and quadriplegia. FoIlow-up brain MRI showed newly developed acute ischemic lesions at both parietal area MR venography confirmed superior sagittal sinus thrombosis. Methionine and homocystine were markedly elevated in plasma and 24 hour urine. She recovered with anticoagulation and vitamin supplementation(folate and pyridoxine). Homocystinuria should be suspected in stroke patients of young age, especially if thy have nontraumatic lens dislocation or marfanoid features. We report a patient with homocystinuria complicated by cerebral venous sinus thrombosis which was aggrevated after cerebral angiography.
Adolescent ; Angiography ; Biopsy ; Brain ; Carotid Artery, Internal ; Cerebral Angiography ; Congenital Abnormalities ; Constriction, Pathologic ; Female ; Homocystine ; Homocystinuria* ; Humans ; Intellectual Disability ; Lens Subluxation ; Magnetic Resonance Imaging ; Metabolism ; Methionine ; Phlebography ; Plasma ; Quadriplegia ; Rabeprazole ; Scoliosis ; Seizures ; Sinus Thrombosis, Intracranial ; Stroke ; Stupor ; Superior Sagittal Sinus* ; Thrombosis* ; Vascular Diseases ; Vitamins

Background: By applying the suggested criteria for needs-based chronic medical care and long-term care delivery system for the elderly, the current status of delivery system was identified and regional delivery systems were categorized according to quantity and quality of delivery system. Methods: National claims data were used for this study. All claims data of medical and long-term care uses by the elderly and all claims data from long-term care hospitals and nursing homes in 2016 were analyzed to categorize the regional medical and long-term care delivery system. The current status of the delivery system with a high possibility of transition to a needs-based appropriate delivery system was identified. The necessary and actual amount of regional supply was calculated based on their needs, and the structure of delivery systems was evaluated in terms of the needs-based quality of the system. Finally, all regions were categorized into 15 types of medical and care delivery systems for the elderly. Results: Of the total 55 regions, 89.1% of regions had an oversupply of elderly medical and care services compared to the necessary supply based on their needs. However, 69.1% of regions met the criteria for less than two types of needs groups, and 21.8% of regions were identified as regions where the numbers of institutions or regions with a high possibility of transition to an appropriate delivery system were below the average levels for all four needs groups. Conclusion In order to establish an appropriate community-based integrated elderly care system, it is necessary to analyze the characteristics of the regional delivery system categories and to plan a needs-based delivery system regionally.

Phytochemicals were isolated from leaves of the fiber crop, ramie (Boehmeria nivea, Bn), using open column chromatography and medium pressure liquid chromatography. Their structures were identified as β-sitosterol, (-)-loliolide, rutin, and pyrimidinedione by MS, ¹H-, and ¹³C-NMR spectroscopic analysis. Among them, (-)-loliolide was isolated for the first time from B. nivea. A content analysis of (-)-loliolide in B. nivea collected from different regions and harvest times was conducted by HPLC. The highest content of (-)-loliolide was found in Bn-23 harvested in September. These results will be helpful to use the plant which harvest in September as a high content phytochemical additive in food, health supplements, and medicinal products.
Boehmeria* ; Chromatography ; Chromatography, High Pressure Liquid* ; Chromatography, Liquid ; Phytochemicals ; Plants ; Rutin ; Urticaceae

BACKGROUND: An increase in the obese adolescent population is being recognized as a serious medical and social problem. The present study aimed to examine the association between neighborhood socioeconomic status (SES) and obesity in Korean adolescents based on total available resources and local social inequality models. METHODS: The present study used data from the 2013 Korea Youth Risk Behavior Web-based Survey in analyzing 72,438 Korean adolescents aged 12-18. The analysis investigated obesity odds ratio (OR) according to neighborhood SES adjusted for age and individual SES indices, which included family affluence scale (FAS), education level of parents, cohabitation with parents, and weekly allowance. Obesity OR was investigated according to neighborhood SES by FAS, and according to FAS by neighborhood SES. RESULTS: After adjusting for age and individual SES variables, there was no significant association between neighborhood SES and adolescent obesity for either boys or girls. However, girls in the high FAS group showed a pattern of lower neighborhood SES being associated with a significant increase in risk of obesity; in the high neighborhood SES group, boys showed a pattern of higher FAS being associated with a significant increase in risk of obesity, whereas girls show a pattern of decrease. CONCLUSION: Although limited, the present study demonstrated that some girl groups exhibited a pattern of lower neighborhood SES being associated with an increase in risk of obesity, as well as a gender-based difference in risk of obesity by individual SES. Therefore, measures to prevent adolescent obesity should be established with consideration for differences in risk according to individual and neighborhood SES.
Adolescent* ; Education ; Female ; Humans ; Korea* ; Obesity* ; Odds Ratio ; Parents ; Pediatric Obesity ; Residence Characteristics* ; Risk-Taking* ; Social Class* ; Social Problems ; Socioeconomic Factors

We report the first Korean patient with familial hemiplegic migraine type 1, with clinical and multimodal imaging findings. A 43-yr-old man was admitted for right hemianopia and aphasia, followed by coma. MRI showed only cerebellar atrophy. CT angiography showed mild vasodilation of intracranial blood vessels and increased vascularity in the left hemisphere and perfusion-weighted imaging showed elevated cerebral blood flow. Gene analysis of the patient and his mother led to the identification of a heterozygous point mutation (1997C-->T, T666M) in exon 16 of the CACNA1A gene. Familial hemiplegic migraine should be considered in patients with episodic neurological dysfunction with cerebellar atrophy.
Asian Continental Ancestry Group/*genetics ; Atrophy/genetics/metabolism ; Calcium Channels/*genetics ; Cerebellum/blood supply/*pathology ; Cerebral Angiography ; Coma/*diagnosis ; Exons ; Heterozygote ; Humans ; Magnetic Resonance Imaging ; Male ; Migraine with Aura/*diagnosis/genetics ; Point Mutation ; Republic of Korea ; Tomography, X-Ray Computed

Phytochemical analysis of Boehmeria nivea (Bn) leaves by medium pressure liquid chromatography led to the isolation of a flavonoid glycoside identified by spectroscopic analysis as rutin. The amount of rutin in the leaves of Bn harvested from nine regions in South Korea (Bn 1–9) which were collected on the months of June, July, August, and September was determined by HPLC-UV analysis. A gradient elution program that utilizes a Discovery® C18 (4.6 × 250 mm, 5 µm) column and mobile phase composed of 1% acetic acid-water: acetonitrile (90:10 to 60:40 for min) was followed. The injection volume and flow rate were 10 µl and 1 mL/ min, respectively. UV detection was set at 350 nm. Results show that Bn-8 harvested in September reported the highest content of rutin among the samples analyzed. This study provides a basis for the optimal harvest time of Bn which maximizes the yield of rutin.
Boehmeria ; Chromatography, Liquid ; Korea ; Rutin

Background: Shift work, including night shift work, during pregnancy has been associated with adverse birth outcomes such as small for gestational age (SGA) infants and preterm births. This study, conducted in South Korea using the Korean CHildren’s ENvironmental health Study (Ko-CHENS) cohort, aimed to investigate the association between shift work and night shift status during pregnancy and adverse birth outcomes. Methods: The Korean Ko-CHENS is a nationwide prospective birth cohort study of children’s environmental diseases, conducted by the Ministry of Environment and the National Institute of Environmental Research. This study included pregnant women recruited from 2015 to 2020 for Ko-CHENS Core Cohorts, and 4,944 out of a total of 5,213 pregnant women were selected as final subjects. A logistic regression model was used to identify the risk factors affecting SGA births, preterm births, and low-birth-weight infants, and the odds ratio (OR) was adjusted.This was confirmed by calculating ORs. Maternal age, infant sex, maternal educational status, body mass index, smoking status, alcohol consumption status, parity, gestational diabetes mellitus, preeclampsia, and abortion history were used as adjusted variables. Results: No statistically significant differences were observed in the birth outcomes or maternal working patterns. There were no significant differences in the adjusted odds ratios (aORs) of SGA and preterm births between the non-worker, day worker, and shift worker.However, there was a significant difference in the aORs of SGA between non-workers and night shift workers. (aORs [95% confidence interval], 2.643 [1.193–5.859]). Conclusion Working during pregnancy did not increase the risk of SGA or preterm birth, and night shift work did not increase the risk of preterm birth. However, night-shift work increases the risk of SGA.

Bowel ischemia following embolization is the major complication of arterial embolization in gastrointestinal bleeding. Although mild ischemia recovers with conservative treatment, perforation should be treated surgically. We report a case of gastrointestinal stromal tumor mistaken for an abscess following embolization of superior mesenteric artery. A 72-year-old female was referred to the emergency room complaining of hematochezia and hematemesis. Endoscopic examination revealed a tiny mucosal defect with spurting bleeding at distal duodenum. Hemostasis could be achieved by embolization of superior mesenteric artery. After ten days, abdominal CT scan showed an abscess-like feature around distal duodenum, but the small bowel series did not show leakage of contrast. Endoscopy revealed round intestinal wall defect. She underwent laparotomy owing to the possibility of perforation and abscess, but surgical findings showed 5 cm mass based on the jejunum just caudal to ligament of Treitz. The mass was finally diagnosed as gastrointestinal stromal tumor combined with necrosis.
Abscess ; Aged ; Duodenum ; Emergencies ; Endoscopy ; Female ; Gastrointestinal Hemorrhage ; Gastrointestinal Stromal Tumors ; Hematemesis ; Hemorrhage ; Hemostasis ; Humans ; Ischemia ; Jejunum ; Laparotomy ; Ligaments ; Mesenteric Artery, Superior ; Necrosis

Bowel ischemia following embolization is the major complication of arterial embolization in gastrointestinal bleeding. Although mild ischemia recovers with conservative treatment, perforation should be treated surgically. We report a case of gastrointestinal stromal tumor mistaken for an abscess following embolization of superior mesenteric artery. A 72-year-old female was referred to the emergency room complaining of hematochezia and hematemesis. Endoscopic examination revealed a tiny mucosal defect with spurting bleeding at distal duodenum. Hemostasis could be achieved by embolization of superior mesenteric artery. After ten days, abdominal CT scan showed an abscess-like feature around distal duodenum, but the small bowel series did not show leakage of contrast. Endoscopy revealed round intestinal wall defect. She underwent laparotomy owing to the possibility of perforation and abscess, but surgical findings showed 5 cm mass based on the jejunum just caudal to ligament of Treitz. The mass was finally diagnosed as gastrointestinal stromal tumor combined with necrosis.
Abscess ; Aged ; Duodenum ; Emergencies ; Endoscopy ; Female ; Gastrointestinal Hemorrhage ; Gastrointestinal Stromal Tumors ; Hematemesis ; Hemorrhage ; Hemostasis ; Humans ; Ischemia ; Jejunum ; Laparotomy ; Ligaments ; Mesenteric Artery, Superior ; Necrosis