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.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

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