Neurofibromatosis(von Reckling hausen's disease) is a congenital heredofamilial disorderresulting from dysplasia of neuroectodermal and mesodermal tissues. It is characterized by developmental anormaly, pigmentary skin lesions and multiple tumors derived from the Schwannian cells of the peripheral and sensory nerve. The authors have experienced two cases of Neurofibromatosis. which is clinically diagnosed. and confirmed by histopathological examination. It was reviewed clinically with the literature.
Mesoderm ; Neural Plate ; Neurofibromatoses* ; Skin

OBJECTIVE: The present study was performed to investigate whether apoptosis occur in human embryos by annexin staining and detect the expression of Fas, Fas-ligand (FasL), Bax, and Bcl-2 in human fragmented embryos derived from IVF-ET by immunofluorescence and Western blot analysis. MATERIALS AND METHODS: Using annexin staining, immunofluorescence and Western blot analysis on normal and fragmented embryos, we were able to detect apoptotsis and apoptotic gene products in fragmented embryos. RESULTS: Phosphatidylserine (PS) translocation, the marker for apoptosis, were detected frequently in fragmented embryos. Bcl-2 and Bax protein were detected in both fragmented and non-fragmented embryos. When fragmented embryos compared to normal embryos, immunofluorescent intensity of Bcl-2 tended to be lower in fragmented embryos. Bax gene expression increased in the fragmented embryos compared to the normal embryos. This result supports a model in which the molar ratio of Bcl-2 to Bax determines whether apoptosis induced or inhibited in human embryo. Fas was highly expressed in human preimplantation embryos but not FasL. It suggests that embryo may undergo apoptosis by binding with FasL produced by follicular or immune cells. CONCLUSION: The over expression of Bax and Fas will trigger apoptosis to lead embryo fragmentation and change embryo to be nonviable.
Apoptosis* ; bcl-2-Associated X Protein ; Blastocyst ; Blotting, Western ; Embryonic Structures* ; Fluorescent Antibody Technique ; Gene Expression ; Humans* ; Molar

OBJECTIVES: The objectives of this study were to estimate the continuity of care for all Koreans with diabetes and to identify factors affecting the continuity of care. METHODS: We obtained National Health Insurance claims data for patients with diabetes who visited health-care providers during the year 2004. A total of 1,498,327 patients were included as study subjects. Most Frequent Provider Continuity (MFPC) and Modified, Modified Continuity Index (MMCI) were used as indexes of continuity of care. A multiple linear regression analysis was used to identify factors affecting continuity of care. RESULTS: The average continuity of care in the entire population of 1,498,327 patients was 0.89+/-0.17 as calculated by MFPC and 0.92+/-0.16 by MMCI. In a multiple linear regression analysis, both MFPC and MMCI were lower for females than males, disabled than non-disabled, Medicaid beneficiaries than health insurance beneficiaries, patients with low monthly insurance contributions, patients in rural residential areas, and patients whose most frequently visited provider is the hospital. CONCLUSIONS: The continuity of care for patients with diabetes is high in Korea. However, women, the disabled and people of low socio-economic status have relatively low continuity of care. Therefore, our first priority is to promote a diabetes management program for these patients.
Poverty ; National Health Programs ; Middle Aged ; Medical Assistance ; Male ; Linear Models ; Korea/epidemiology ; Insurance Claim Review ; Humans ; Female ; Diabetes Mellitus/economics/epidemiology/*therapy ; Continuity of Patient Care/economics/*statistics & numerical data ; Aged ; Adult

The crown-rump length(CRL) remains the most accurate parameter used for gestational dating. We aimed to establish the early fetal growth with CRL range in Korean women. The CRL of 48 singleton pregnancies which resulted from in vitro fertilization and e mbryo transfer(IVF-ET) at SNUH were assessed two to six times in the first trimester by trans-vaginal ultrasonography. All women included in this study went on to deliver norma l infa-nts at 37 completed weeks or after weighing over 2.5 kg. And we also studied the r elationship between CRL(in millimeters) and gestational age(in days), and found that the fo llowing second-order polynomial might be applied either to expect CRL using the menstr ual gestational age, or to estimate gestational age using measured CRL(r2=0.980, p=0.000 1). CRL=0.0175(GA)2-1.049(GA)+19.17 GA=7.5593(CRL-3.45)1/2+29.97 Finally we compared our data with some of published articles which assessed CRL in spontaneous and induced pregnancies. In conclusion, this study establish the early fetal growth with CRL range in Korean women on the basis of exact ovulation timing using high resolution transvaginal ultrasonography. And these data will be of great use in the evaluation of fetal growth in the first trimester.
Crown-Rump Length* ; Embryo Transfer* ; Embryonic Structures* ; Female ; Fertilization in Vitro* ; Fetal Development ; Gestational Age ; Humans ; Ovulation ; Pregnancy ; Pregnancy Trimester, First* ; Ultrasonography

Objective: To develop and evaluate a deep learning model for automated segmentation and detection of bone metastasis on spinal MRI. Materials and Methods: We included whole spine MRI scans of adult patients with bone metastasis: 662 MRI series from 302 patients (63.5 ± 11.5 years; male:female, 151:151) from three study centers obtained between January 2015 and August 2021 for training and internal testing (random split into 536 and 126 series, respectively) and 49 MRI series from 20 patients (65.9 ± 11.5 years; male:female, 11:9) from another center obtained between January 2018 and August 2020 for external testing. Three sagittal MRI sequences, including non-contrast T1-weighted image (T1), contrast-enhanced T1-weighted Dixon fat-only image (FO), and contrast-enhanced fat-suppressed T1-weighted image (CE), were used. Seven models trained using the 2D and 3D U-Nets were developed with different combinations (T1, FO, CE, T1 + FO, T1 + CE, FO + CE, and T1 + FO + CE). The segmentation performance was evaluated using Dice coefficient, pixel-wise recall, and pixel-wise precision. The detection performance was analyzed using per-lesion sensitivity and a free-response receiver operating characteristic curve. The performance of the model was compared with that of five radiologists using the external test set. Results: The 2D U-Net T1 + CE model exhibited superior segmentation performance in the external test compared to the other models, with a Dice coefficient of 0.699 and pixel-wise recall of 0.653. The T1 + CE model achieved per-lesion sensitivities of 0.828 (497/600) and 0.857 (150/175) for metastases in the internal and external tests, respectively. The radiologists demonstrated a mean per-lesion sensitivity of 0.746 and a mean per-lesion positive predictive value of 0.701 in the external test. Conclusion The deep learning models proposed for automated segmentation and detection of bone metastases on spinal MRI demonstrated high diagnostic performance.

PURPOSE: The transoral endoscopic thyroid surgery vestibular approach (TOETVA) is one of the newest techniques which do not result in an anterior neck scar. We report herein our initial experience with TOETVA and its short-term outcomes in patients with papillary thyroid carcinoma (PTC). METHODS: This case series consisted of all consecutive patients who underwent TOETVA in our institution between August 2016 and June 2017. Indications for TOETVA were an fine needle aspiration-confirmed PTC or follicular neoplasm, an intrathyroidal tumor with a diameter of less than 2 cm, and no clinical evidence of central or lateral lymph node metastasis. A total of 20 patients underwent TOETVA and we retrospectively reviewed clinicopathologic data and short-term postoperative outcomes. RESULTS: Among the 20 TOETVA cases, 7 were total thyroidectomy, 12 were lobectomy and 1 was wide isthmusectomy. Mean age was 50.8 years and mean operation time was 152 ± 51.4 minutes. Tumor size ranged from 0.2–1.4 cm and the mean size of harvested central lymph nodes was 2.8 (range, 0–10). Neither mental nerve injury nor surgical site infection occurred. One patient had transient vocal cord palsy and 1 patient developed a neck seroma. Among 7 total thyroidectomy patients, 3 patients developed transient hypocalcemia. CONCLUSION: Transoral thyroid surgery could be an alternative surgical option for some PTC patients.
Cicatrix ; Endoscopy ; Humans ; Hypocalcemia ; Korea* ; Lymph Nodes ; Neck ; Needles ; Neoplasm Metastasis ; Retrospective Studies ; Seroma ; Surgical Wound Infection ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy ; Vocal Cord Paralysis

PURPOSE: The purpose of this study was to present a technique for measuring physiologic distal tibiofibular syndesmosis widening using 3-dimensional ultrasonography (3D-US) with an evaluation of its reliability, and to determine whether there were differences in the measurements between different dynamic stress tests. METHODS: We retrospectively evaluated 3D-US of 20 subjects with normal ankle syndesmosis. 3D-US was performed in neutral (N), dorsiflexion with external rotation (DFER), and weight-bearing standing (WB) positions at the anterior inferior tibiofibular ligament level in both ankles for comparison. Using 3D-US volume data, axial images were reconstructed at the level of the lateral prominence of the anterior tibial tubercle to ensure consistent measurements of the tibiofibular clear space (TFCS) by two radiologists. RESULTS: There was a wide range of TFCS values among the subjects (N, 1.2 to 4.2 mm; DFER, 2.3 to 4.8 mm; WB, 1.7 to 4.6 mm). When both ankles of each subject were evaluated, the side-to-side differences were less than 1 mm in all positions, with high intraclass correlation coefficient (ICC) values between both ankles (ICC, 0.85 to 0.93). The inter-rater agreement for all TFCS measurements between the two radiologists was excellent (ICC, 0.81 to 0.96). In comparisons between the two dynamic stress tests, the TFCS was significantly wider in the DFER position than in the WB position (DFER vs. WB, 3.3 mm vs. 2.9 mm; P<0.001). CONCLUSION: Using 3D-US, we were able to consistently evaluate the TFCS with good reliability. In a comparison of the two dynamic tests, there was more significant widening of the TFCS in the DFER position than in the WB position.
Ankle Joint ; Ankle ; Diagnosis ; Exercise Test ; Lateral Ligament, Ankle ; Retrospective Studies ; Ultrasonography ; Weight-Bearing

Purpose: The aim of this study was to compare the large multi-institutional data of surgical outcomes of bilateral axillo-breast approach (BABA) robotic (RT) and endoscopic thyroidectomy (ET) and to evaluate the merits of robotic thyroidectomy. Methods: From 2004 to 2015, 1,029 patients underwent BABA ET, and from 2008 to 2015, 2003 patients underwent BABA RT in 3 large volume centers in Korea. Two groups were retrospectively compared in terms of clinicopathologic characteristics, complications, surgical completeness, and long-term outcomes using propensity score matching analysis. Results: Both groups had similar demographic characteristics including age, sex, tumor size, pathologic stage, and hospital stay after matching. Each group had similar rate of transient hypoparathyroidism, however, ET showed significantly higher rate of permanent hypoparathyroidism (ET 5.2% vs. RT 2.3%, P = 0.05), and transient vocal cord palsy (ET 14.4% vs. RT 9.1%, P = 0.006). Total operation time was longer in the ET group irrespective of surgical extents, including lobectomy (P = 0.016), total thyroidectomy (P = 0.031), and total thyroidectomy with central lymph node dissection (P = 0.019). The rate of patients with off-Tg under 1.0 ng/mL after 1st ablation was significantly higher in RT than ET group (ET 64.6% vs. RT 92.7%, P < 0.001). In long-term follow-up of cancer patients, 1.4% experienced recurrence after ET (10 cases), while 0.3% cases experienced recurrence after RT (5 cases) (P < 0.001). Conclusion Both ET and RT can be safe and effective methods to treat thyroid diseases. However, the application of robotic system may help to overcome the limitations of the instruments and surgeon’s skills.

Background/Aims: The aim of this study was to investigate long-term post-discontinuation outcomes in patients with rheumatoid arthritis (RA) who had been treated with tumor necrosis factor-α inhibitors (TNF-αi) which was then discontinued. Methods: Sixty Korean patients with RA who participated in a 5-year GO-BEFORE and GO-FORWARD extension trials were included in this retrospective study. Golimumab was deliberately discontinued after the extension study (baseline). Patients were then followed by their rheumatologists. We reviewed their medical records for 2 years (max 28 months) following golimumab discontinuation. Patients were divided into a maintained benefit (MB) group and a loss-of-benefit (LB) group based on treatment pattern after golimumab discontinuation. The LB group included patients whose conventional disease-modifying antirheumatic drug(s) were stepped-up or added/switched (SC) and those who restarted biologic therapy (RB). Results: The mean age of patients at baseline was 56.5 years and 55 (91.7%) were females. At the end of follow-up, 23 (38.3%) patients remained in the MB group. In the LB group, 75.7% and 24.3% were assigned into SC and RB subgroups, respectively. Fifty percent of patients lost MB after 23.3 months. Demographics and clinical variables at baseline were comparable between MB and LB groups except for age, C-reactive protein level, and corticosteroid use. Restarting biologic therapy was associated with swollen joint count (adjusted hazard ratio [HR], 1.90; 95% confidence interval [CI], 1.01 to 3.55) and disease duration (adjusted HR, 1.12; 95% CI, 1.02 to 1.23) at baseline. Conclusions Treatment strategies after discontinuing TNF-αi are needed to better maintain disease control and quality of life of patients with RA.

BACKGROUND: Aldosterone antagonists are reported to have beneficial effects on diabetic nephropathy by effective blocking of the renin-angiotensin-aldosterone system. We investigated the renoprotective effect of the selective aldosterone receptor blocker eplerenone, the angiotensin converting enzyme inhibitor lisinopril, and combined eplerenone and lisinopril treatment in type 2 diabetic rats. METHODS: Animals were divided into six groups as follows: Otsuka Long-Evans Tokushima Fatty (OLETF) rat control, OLETF rats treated with a low dose of eplerenone (50 mg/kg/day), OLETF rats treated with a high dose of eplerenone (200 mg/kg/day), OLETF rats treated with lisinopril (10 mg/kg/day), OLETF rats treated with a combination of both drugs (eplerenone 200 mg/kg/day and lisinopril 10 mg/kg/day), and obese non-diabetic Long-Evans Tokushima Otsuka rats for 26 weeks. RESULTS: Urinary albumin excretion was significantly lower in the lisinopril group, but not in the eplerenone group. Urinary albumin excretion was decreased in the combination group than in the lisinopril group. Glomerulosclerosis and renal expression of type I and type IV collagen, plasminogen activator inhibitor-1, transforming growth factor-beta1, connective tissue growth factor, and fibronectin mRNA were markedly decreased in the lisinopril, eplerenone, and combination groups. CONCLUSION: Eplerenone and lisinopril combination showed additional benefits on type 2 diabetic nephropathy compared to monotherapy of each drug.
Aldosterone ; Animals ; Collagen Type IV ; Connective Tissue Growth Factor ; Diabetic Nephropathies ; Fibronectins ; Lisinopril ; Mineralocorticoid Receptor Antagonists ; Peptidyl-Dipeptidase A ; Plasminogen Activators ; Rats ; Rats, Inbred OLETF ; Receptors, Mineralocorticoid ; Renin-Angiotensin System ; RNA, Messenger ; Spironolactone