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

A 75-year-old male with recurrent pancreatic adenocarcinoma after a previous Whipple's procedure presented with jaundice. The local advancement of the tumor caused obstructions of the common bile duct, intrahepatic bile duct hilum, and small bowel. Endoscopic stent insertion was precluded by the Roux-en-Y reconstruction. A successful transhepatic percutaneous single-access stenting of the whole biliary tree and intestine was achieved by H-configured triple stenting by combining T-configured dual stent placement in the biliary system with a duodenal stent insertion across the bottom of the anastomosis after looping a wire in the afferent limb. The ‘H-configured’ stents remained patent for 10 months without major or minor complications. This technique adds a new minimal-invasive and effective palliative option for patients with obstruction of a bilio-enteric anastomosis inaccessible to endoscopy.
Adenocarcinoma ; Aged ; Bile Ducts, Intrahepatic ; Biliary Tract ; Cholestasis ; Common Bile Duct ; Endoscopy ; Extremities ; Gastric Outlet Obstruction ; Humans ; Intestines ; Jaundice ; Male ; Stents

Background: This study was conducted to analyze the impact of having a usual source of care on health behaviors of the middle aged and the elderly, in order to investigate the potential effect of enhancing primary health care in a Korean context. Methods: This study constructed a balanced panel of middle-aged and elderly samples using the Korea Health Panel 2016–2018, and fixed-effect models were used to analyze the data. Results: Among three sets of dependent variables (physical activity, smoking, drinking), statistically significant results were found only in physical activity. Subgroup analysis showed that this effect was not observed in the late elderly (aged 75 and older) and those without chronic diseases. Conclusion Results of the study implied that enhancing primary health care among middle age and the elderly may have an effect on improving health behaviors. Moving forward to person-centered primary health care from disease-focused primary health care should be considered in high-risk groups such as the middle aged and the elderly with chronic diseases.

Operative vaginal birth retains an important role in current obstetric practice. However, there is an increasing trend in the rate of cesarean section in Korea. Surgical delivery is more advantageous than cesarean section, but the rate of operative vaginal delivery is decreasing for various reasons. Furthermore, there is no unified technique for vacuum extraction delivery. In this context, this review was performed to provide details of the necessary conditions, techniques, benefits, and risks of operative vaginal delivery. Future research should focus on overcoming the limitations of operative vaginal delivery.
Cesarean Section ; Delivery, Obstetric ; Female ; Korea ; Parturition ; Pregnancy ; Vacuum Extraction, Obstetrical ; Vacuum*

PURPOSE: The purpose of this study is to evaluate the result of primary total knee arthroplasty(TKA) with using NexGen CR-Flex(R) (Zimmer, Warsaw, IN, USA) after a follow-up of at least 2 years. MATERIALS AND METHODS: A total of 63 patients (98 knees) were included in this study. The same surgeon performed all the TKAs between August 2005 and May 2006 with using NexGen CR-Flex(R). The patients' age ranged from 52 to 81 years, and the mean follow-up period was 28 months. The patients were assessed clinically and radiographically with using the HSS score, the possibility that they could squat or kneel, the tibiofemoral angle, the radiolucency and the posterior condylar offset. RESULTS: The mean HSS score was 58.3 points preoperatively, and it was 91.1 points at the last follow-up. The mean range of motion improved from 123.2degrees preoperatively to 137.1degrees postoperatively. Forty-nine patients (78%) were able to squat and 24 patients (38%) could kneel down at the last follow-up. The mean tibiofemoral angle was 6degrees of varus preoperatively, and 7degrees of valgus postoperatively. And there was no radiolucency in any case. The mean posterior condylar offset was 26.1mm preoperatively and 27.2 mm postoperatively. CONCLUSION: TKA with using NexGen CR-Flex(R) showed favorable results at a mean follow-up of 28 months. Adequate patient selection, restoration of the posterior condylar offset and thorough clearing of posterior condylar osteophytes are thought to be the key elements to achieve a satisfactory range of motion after TKAs.
Arthroplasty ; Follow-Up Studies ; Humans ; Knee ; Knee Joint ; Osteophyte ; Patient Selection ; Range of Motion, Articular

OBJECTIVE: To examine the correlation among the preoperative serum levels of five biomarkers presumed to be useful for early detection of epithelial ovarian cancer and evaluate the relationships between serum levels of these five biomarkers and epithelial ovarian cancer stage. METHODS: We analyzed 56 newly diagnosed epithelial ovarian cancer patients. Preoperative serum levels of leptin, prolactin, osteopontin (OPN), insulin-like growth factor-II, and CA-125 were determined by ELISA. We also examined the correlation between the serum levels of the biomarkers and ovarian cancer stage. Significant differences in the mean serum levels of two proteins, leptin and CA-125, were observed between stage subsets. RESULTS: There was a significant negative correlation between prolactin and leptin and a significant positive correlation between prolactin and OPN. Of the five biomarkers, only the mean serum CA-125 level showed a significant positive correlation with cancer stage (Spearman rho=0.24, p<0.01). OPN showed a marginally significant positive correlation with stage (Spearman rho=0.14, p=0.07). CONCLUSION: We demonstrated the relationship between five biomarkers in epithelial ovarian cancer. These tumor markers may be useful in screening for ovarian cancer, in characterizing disease states, and in developing therapeutic interventions targeting these marker proteins. Large-scale studies that include potential confounding factors and modifiers are necessary to more accurately define the value of these novel biomarkers in ovarian cancer.
Biomarkers ; Enzyme-Linked Immunosorbent Assay ; Humans ; Leptin ; Mass Screening ; Neoplasms, Glandular and Epithelial ; Osteopontin ; Ovarian Neoplasms ; Prolactin ; Proteins ; Biomarkers, Tumor

This review focused on the research published to date on the treatment of phalangeal fractures according to the anatomical location of the finger bones, excluding the thumb. In many finger fracture cases, conservative treatment should be prioritized over surgical treatment. The three determinants of surgical treatment are the presence of an intra-articular fracture, the stability of the fracture itself, and the degree of damage to the surrounding soft tissues. Surgical treatment is recommended when bone fragments of 3 mm or more and distal phalanx subluxation are present in the bony mallet finger, and the main surgical treatment is closed reduction and extension block pin fixation. It is essential to pay attention to rotational deformation asf ractures occur proximally. Since intra-articular fractures can cause stiffness and arthritis in the future, a computed tomography scan is recommended to confirm the fracture pattern. These fractures require anatomical reduction of the bone fragments within the joint, and the instability of the joint itself must be corrected. There are no superior surgical treatment methods. It is therefore advantageous for the surgeon to select a surgical method that he is familiar with and confident of performing, considering the fracture itself and various patient-related clinical factors. Nonunion is rare as a complication of a finger fracture, and finger stiffness is the most common complication. Ensuring rapid joint movement as soon as possible can reduce finger stiffness.

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.

We report a very high serum level of serum CA 125 in a patient with adenomyosis. Elevated serum CA 125 is useful for the management of patients with ovarian cancer but has also been associated with several benign conditions, including adenomyosis, uterine fibroids, pelvic inflammatory disease, pregnancy, menstruation, and endometriosis. Adenomyosis refers to the endometrial glands and stroma located deep within the myometrium. Here we report a patient with an elevated, rising serum CA 125 level over 1,000 IU/mL without any malignancy. Exploratory laparoscopy with LAVH (Laparoscopically assisted vaginal hysterectomy) and multiple peritoneal biopsies were performed. Histologically, the lesion was confirmed to be an adenomyosis. The level of serum CA 125 was 38 IU/mL on the fifth postoperative day.
Adenomyosis ; Animals ; Biopsy ; Endometriosis ; Female ; Humans ; Laparoscopy ; Leiomyoma ; Menstruation ; Mice ; Myometrium ; Ovarian Neoplasms ; Pelvic Inflammatory Disease ; Pregnancy