Maternally inherited diabetes and deafness (MIDD) is a rare mitochondrial disorder primarily resulting from m.3243A>G mutation. The clinical characteristics of MIDD exhibit significant heterogeneity. Our study aims to delineate these characteristics and determine the potential correlation with m.3243A>G heteroplasmy levels. This retrospective, descriptive study encompassed patients with confirmed m.3243A>G mutation and diabetes mellitus at Seoul National University Hospital. Our cohort comprises 40 patients with MIDD, with a mean age at study enrollment of 33.3±12.9 years and an average % of heteroplasmy of 30.0%± 14.6% in the peripheral blood. The most prevalent comorbidity was hearing loss (90%), followed by albuminuria (61%), seizure (38%), and stroke (33%). We observed a significant negative correlation between % of heteroplasmy and age at diabetes diagnosis. These clinical features can aid in the suspicion of MIDD and further consideration of genetic testing for m.3243A>G mutation.

Purpose: To derive the optimized intraocular lens (IOL) constants from automated and manifest refraction after cataract surgery in Korean patients, and to evaluate whether there is a difference in optimized IOL constants according to the refraction method. Methods: This retrospective multicenter cohort study enrolled 4,103 eyes of 4,103 patients who underwent phacoemulsification and in-the-bag IOL implantation at 18 institutes. Optimized IOL constants for the SRK/T, Holladay, Hoffer Q, and Haigis formulas were calculated via autorefraction or manifest refraction of samples using the same biometry and IOL. The IOL constants derived from autorefraction and manifest refraction were compared. Results: Of the 4,103 eyes, the majority (62.9%) were measured with an IOLMaster 500 followed by an IOLMaster 700 (15.2%). A total of 33 types of IOLs were used, and the Tecnis ZCB00 was the most frequently used (53.0%). There was no statistically significant difference in IOL constants derived from autorefraction and manifest refraction when IOL constants were optimized with a large number of study subjects. On the other hand, optimized IOL constants derived from autorefraction were significantly smaller than those from manifest refraction when the number of subjects was small. Conclusions It became possible to use the IOL constants optimized from Koreans to calculate the IOL power. However, if the IOL constant is optimized using autorefraction in a small sample group, the IOL constant tends to be small, which may lead to refractive error after surgery.

Purpose: To assess patient radiation doses during diagnostic and therapeutic neurointerventional procedures from multiple centers and propose dose reference level (RL). Materials and Methods: Consecutive neurointerventional procedures, performed in 22 hospitals from December 2020 to June 2021, were retrospectively studied. We collected data from a sample of 429 diagnostic and 731 therapeutic procedures. Parameters including dose-area product (DAP), cumulative air kerma (CAK), fluoroscopic time (FT), and total number of image frames (NI) were obtained. RL were calculated as the 3rd quartiles of the distribution. Results: Analysis of 1160 procedures from 22 hospitals confirmed the large variability in patient dose for similar procedures. RLs in terms of DAP, CAK, FT, and NI were 101.6 Gy·cm2, 711.3 mGy, 13.3 minutes, and 637 frames for cerebral angiography, 199.9 Gy·cm2, 3,458.7 mGy, 57.3 minutes, and 1,000 frames for aneurysm coiling, 225.1 Gy·cm2, 1,590 mGy, 44.7 minutes, and 800 frames for stroke thrombolysis, 412.3 Gy·cm2, 4,447.8 mGy, 99.3 minutes, and 1,621.3 frames for arteriovenous malformation (AVM) embolization, respectively. For all procedures, the results were comparable to most of those already published. Statistical analysis showed male and presence of procedural complications were significant factors in aneurysmal coiling. Male, number of passages, and procedural combined technique were significant factors in stroke thrombolysis. In AVM embolization, a significantly higher radiation dose was found in the definitive endovascular cure group. Conclusion Various RLs introduced in this study promote the optimization of patient doses in diagnostic and therapeutic interventional neuroradiology procedures. Proposed 3rd quartile DAP (Gy·cm2) values were 101.6 for diagnostic cerebral angiography, 199.9 for aneurysm coiling, 225.1 for stroke thrombolysis, and 412.3 for AVM embolization. Continual evolution of practices and technologies requires regular updates of RLs.

BACKGROUND: The study investigated in detail the current status of the consultations requested in a pain clinic. We evaluated the characteristics of the consultations to determine the kind of contents requested, referring departments and factors including demographics, co-morbidities, previous medical problems, and the descriptions of the reasons for the consultation to the pain clinic. METHODS: Clinical data were collected in the authors' institution between 1 January 2009 and 31 December 2013. The medical records were reviewed and compared. Characteristics of both outpatients and inpatients were analysed. RESULTS: Data from 1,140 patients was available for this study. Seven hundred thirteen individuals belonged to the outpatient group and 427 individuals belonged to the inpatient group. Orthopedic surgery, neurosurgery, and otolaryngology were the main departments that requested consultations to the pain clinic. The most frequent requested lesion and diagnostic term were low back and lumbar spinal stenosis, respectively, and the most common reason for consulting was for "control of pain not controlled by medications." Factors that were significantly different between the two groups were gender, questions about other illnesses apart from the main diagnoses, history of specific diseases, acute onset, cancer, operation within 3 months, and physical system abnormalities. CONCLUSIONS: The medical problems addressed by a pain clinic consultation service were diverse. It is rational to develop standardized guidelines for pain consultations, and treatment strategies aimed at alleviating pain per se as well as caring for comorbid conditions.
Acute Disease ; Demography ; Diagnosis ; Humans ; Inpatients ; Medical Records ; Neurosurgery ; Orthopedics ; Otolaryngology ; Outpatients ; Pain Clinics* ; Referral and Consultation* ; Spinal Stenosis

No abstract available.
Humans ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroid Nodule*

No abstract available.
Humans ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroid Nodule*

No abstract available.
Advanced Cardiac Life Support* ; Cardiopulmonary Resuscitation*

PURPOSE: The International Subarachnoid Aneurysm Trial (ISAT) revealed that in ruptured intracranial aneurysms (RA), endovascular coiling (EC) yields better clinical outcomes than neurosurgical clipping (NC) at 1 year. In unruptured aneurysms (UIA), EC is being increasingly used as an alternative to NC due to patients' preference. There is a lot of difference in treatment cost (EC vs. NC) between countries. There is one recently published study dealing with the comparative cost analysis only in UIAs in South Korea. But it is a hospital-based study. So, the authors performed a nation-wide cost effective comparison in our country. MATERIALS AND METHODS: This study was a retrospective analysis of healthcare big data open systems in Health Insurance Review & Assessment Service (HIRA). Hospital cost data of the recent 5 years (from January 2010 to December 2014) were analyzed according to patients' age and sex and the presence of subarachnoid hemorrhage. RESULTS: When comparing the total hospital costs for NC of a UIA (n=13,756) and EC of a UIA (n=17,666), NC [mean±standard deviation (SD): ￦7,987,179±3,855,029] resulted in significantly lower total hospital costs than EC [￦10,201,645±5,001,626, p<0.0001], although a shorter hospital stay with EC of a UIA [8.6 ±7.4 days] vs. NC [15.0 ±8.3 days, p<0.0001]. When comparing the total hospital costs for NC of a RA (n=7,293) and EC of a RA (n=6,954), NC [￦13,914,993±6,247,914] resulted in significantly lower total hospital costs than EC [￦16,702,446±7,841,141, p<0.0001], although shorter hospital stays for EC of a RA [19.8 ±11.4] vs. NC [23.0 ±10.3, p<0.0001]. CONCLUSION: The total hospital costs for the NC of both UIAs and RAs were found to be lower than those for EC in South Korea.
Aneurysm ; Cost-Benefit Analysis* ; Costs and Cost Analysis ; Delivery of Health Care ; Health Care Costs ; Hospital Costs ; Insurance ; Insurance, Health ; Intracranial Aneurysm* ; Korea ; Length of Stay ; Republic of Korea* ; Retrospective Studies ; Subarachnoid Hemorrhage

PURPOSE: Pediatric-type sarcomas such as rhabdomyosarcoma (RMS), Ewing sarcoma (EWS), primitive neuroectodermal tumor (PNET), and desmoplastic small round-cell tumor (DSRCT) are rare in adults, with limited studies on their prognosis and optimal treatment strategies. We aimed to examine the outcome of children and adult patients with RMS, EWS, PNET, and DSRCT and relevant prognostic factors. MATERIALS AND METHODS: We retrospectively reviewed 220 pediatric-type sarcoma patients at a single institution between 1985 and 2011. Comparisons were made in order to examine differences in demographics, disease characteristics, and survival. Survival analyses were performed using the Kaplan-Meier method with log-rank tests and Cox proportional hazards models. RESULTS: A total of 220 consecutive patients were identified at our institute. Median age was 15.6 years (range, 0 to 81 years) and there were 108 children (49%) and 112 adult patients (51%). According to histological classification, 106 patients (48.2%) had RMS, 60 (27.3%) had EWS, 50 (22.7%) had PNET, and 4 (1.8%) had DSRCT. With a median follow-up period of 6.6 years, the estimated median overall survival (OS) of all patients was 75 months (95% confidence interval [CI], 27.2 to 122.8 months) and median event-free survival (EFS) for all patients was 11 months (95% CI, 8.8 to 13.2 months). No significant difference in OS and EFS was observed between adults and children. In multivariate analysis, distant metastasis (hazard ratio [HR], 1.617; 95% CI, 1.022 to 2.557; p=0.040) and no debulking surgery (HR, 1.443; 95% CI, 1.104 to 1.812; p=0.012) showed independent association with worse OS. CONCLUSION: Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.
Adult* ; Child* ; Classification ; Demography ; Desmoplastic Small Round Cell Tumor ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Incidence* ; Multivariate Analysis ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive ; Prognosis ; Proportional Hazards Models ; Retrospective Studies ; Rhabdomyosarcoma ; Sarcoma* ; Sarcoma, Ewing

PURPOSE: To compare the macular choroidal thickness, ganglion cell complex thickness, peripapillary choroidal thickness and retinal nerve fiber layer thickness among normal, primary open angle glaucoma (POAG) and normal tension glaucoma (NTG) patients using RTVue (Fourier-domain optical coherence tomography; Optovue, Fremont, CA, USA). METHODS: A retrospective analysis of 32 normal controls, 32 POAG and 52 NTG patients was performed. Choroidal thickness, ganglion cell complex thickness and retinal nerve fiber layer thickness were compared among normal controls, POAG and NTG subjects. Additionally, the factors influencing choroidal thickness (age, axial length, spherical equivalent, central corneal thickness, mean deviation, nocturnal dip, blood pressure variability) were analyzed. RESULTS: A total of 32 normal controls, 32 POAG and 52 NTG patients were enrolled in this study. Macular and peripapillary choroidal thicknesses were significantly thinner in the NTG patients. In NTG subjects, the significant influencing factors associated with macular and peripapillary choroidal thicknesses were age, axial length, nocturnal dip (diastolic blood pressure), diastolic blood pressure variability and ganglion cell complex thickness. In POAG patients, significant influencing factors associated with macular and peripapillary choroidal thicknesses were age and axial length. CONCLUSIONS: Choroidal thickness was significantly thinner in NTG patients compared with normal controls and POAG patients. Factors influencing choroidal thickness in NTG patients were age, axial length, nocturnal dip (diastolic blood pressure), diastolic blood pressure variability and ganglion cell complex thickness. In POAG patients, significant factors influencing choroidal thickness were age and axial length.
Blood Pressure ; Choroid* ; Ganglion Cysts ; Glaucoma, Open-Angle* ; Humans ; Low Tension Glaucoma ; Nerve Fibers ; Retinaldehyde ; Retrospective Studies ; Tomography, Optical Coherence