Fahr's disease (FD) is a rare neurologic disorder characterized by the symmetric and bilateral intracerebral calcification in a patient. We describe the case of a 65-year-old woman who presented with gait disturbance, abnormal mentality, and visual field defect. The result of a brain computerized tomography showed spontaneous intracranial hemorrhage in the right parieto-occipital area, and also showed the incidence of symmetric and bilateral intracerebral calcification. Moreover, laboratory studies indicated characteristic hypoparathyroidism. This brings us to understand that additionally, one of her sons also presented with similar intracerebral calcification, and was subsequently diagnosed with FD. Thus, her case was consistent with that of a patient experiencing FD. The patient had hypertension, which we now know might have caused the intracerebral hemorrhage. However, this patient's brain lesions were in uncommon locations for spontaneous intracerebral hemorrhage, and the lesions were noted as occurring away from the identified heavily calcified areas. Thus, it seemed that the massive calcification of cerebral vessels in the basal ganglia, the most common site of intracerebral hemorrhage, might have prevented a hypertensive intracerebral hemorrhage. Eventually, an intracerebral hemorrhage occurred in an uncommon location in the patient's brain.
Aged ; Basal Ganglia ; Brain ; Cerebral Hemorrhage ; Female ; Gait ; Humans ; Hypertension ; Hypoparathyroidism ; Incidence ; Intracranial Hemorrhage, Hypertensive ; Intracranial Hemorrhages ; Nervous System Diseases ; Visual Fields

OBJECTIVE: Heat therapy is one of physical therapies used most commonly in chronic osteoarthritis. The therapeutic effects of heat therapy might be attributed to induce heat shock proteins in heat-stimulated cells and tissues and therefore, to inhibit cellular damages due to inflammation. In order to investigate preliminarily the therapeutic effects of heat therapy, Hsp(heat shock protein) 70 expressions by heat stimulation were measured in cultured chondrocytes and knee joint cartilages of rabbits. METHOD: Five rabbits were used in total in this study. Three rabbits were used for chodrocytes culture and two rabbits were in vivo study. Chondrocytes were cultured from knee cartilages of three rabbits and treated at 33degreesC, 37degreesC, and 42degreesC for 20 min. In order to clarify whether heat therapy using ultrasonification can induce Hsp 70 expression in cartilage tissues, right knees of rabbits were heat-stimulated by ultrasonification for 20 min and their left knees were untreated. After 2 hours, cultured chondrocytes and cartilages were prepared and Hsp 70 expression was also observed by Western blot analysis. RESULTS: Hsp 70 expression was increased 1.48 folds in 42degreesC treated cells compared to in 37degreesC treated cells. The heat-stimulated cartilages showed 1.65 fold increases in Hsp 70 expression compared to the unstimulated cartilages. CONCLUSION: Hsp 70 expressions were increased by heat stimulation in cultured chondrocytes as well as in cartilage issues.
Blotting, Western ; Cartilage ; Cartilage, Articular* ; Chondrocytes* ; Heat-Shock Proteins* ; Hot Temperature* ; HSP70 Heat-Shock Proteins* ; Inflammation ; Knee ; Knee Joint ; Osteoarthritis ; Rabbits ; Shock

Purpose: Alport syndrome (AS) is one of the most common inherited renal diseases caused due to mutations of genes encoding specific proteins of the type IV collagen family, and its major clinical manifestations include progressive renal failure, sensorineural deafness, and ocular abnormalities. We investigated the clinical characteristics and long-term prognosis of AS in Korean pediatric and adult populations. Methods: We conducted a retrospective review of medical records of 33 children and adults who had been diagnosed or treated with AS from 1985 to 2019. Results: The mean age of the 33 patients diagnosed with AS was 16.2±13.6 years, and the male-to-female ratio was 2:1. At the first visit, recurrent gross hematuria was the most common initial symptom. In 10 of 33 patients (30.3%), sensorineural hearing loss (SNHL) was diagnosed, but none had ophthalmic problems. Moreover, 11 of 33 patients (33.3%) had advanced to end-stage renal disease (ESRD), and a significant difference was observed in the age of the patients who progressed to ESRD based on the presence or absence of SNHL (P =0.035). Conclusion SNHL in AS can be an important prognostic factor for long-term deterioration of renal function. Further investigation is required to confirm the clinical course and the genetic characteristics of AS in Korea through prospective national cohort studies.

We report here on a very rare case of granulocytic sarcoma of the pectoralis muscle on the left chest wall of a patient with chronic myelogenous leukemia, and this malady presented as a very rapidly growing hematoma-like mass.
Humans ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Pectoralis Muscles ; Sarcoma ; Sarcoma, Myeloid ; Thoracic Wall

Retinal prostheses are being developed to restore vision for the blind with retinal diseases such as retinitis pigmentosa (RP) or age-related macular degeneration (AMD). Since retinal prostheses depend upon electrical stimulation to control neural activity, optimal stimulation parameters for successful encoding of visual information are one of the most important requirements to enable visual perception. Therefore, in this paper, we focused on retinal ganglion cell (RGC) responses to different voltage stimulation parameters and compared threshold charge densities in normal and rd1 mice. For this purpose, we used in vitro preparation for the retina of normal and rd1 mice on micro-electrode arrays. When the neural network of rd1 mouse retinas is stimulated with voltage-controlled pulses, RGCs in degenerated retina also respond to voltage amplitude or voltage duration modulation as well in wild-type RGCs. But the temporal pattern of RGCs response is very different; in wild-type RGCs, single peak within 100 ms appears while in RGCs in degenerated retina multiple peaks (~4 peaks) with ~10 Hz rhythm within 400 ms appear. The thresholds for electrical activation of RGCs are overall more elevated in rd1 mouse retinas compared to wild-type mouse retinas: The thresholds for activation of RGCs in rd1 mouse retinas were on average two times higher (70.50~99.87micronC/cm2 vs. 37.23~61.65micronC/cm2) in the experiment of voltage amplitude modulation and five times higher (120.5~170.6micronC/cm2 vs. 22.69~37.57micronC/cm2) in the experiment of voltage duration modulation than those in wild-type mouse retinas. This is compatible with the findings from human studies that the currents required for evoking visual percepts in RP patients is much higher than those needed in healthy individuals. These results will be used as a guideline for optimal stimulation parameters for upcoming Korean-type retinal prosthesis.
Animals ; Electric Stimulation ; Fees and Charges ; Humans ; Macular Degeneration ; Mice ; Retina ; Retinal Diseases ; Retinal Ganglion Cells ; Retinaldehyde ; Retinitis Pigmentosa ; Vision, Ocular ; Visual Perception ; Visual Prosthesis

PURPOSE: The aim of the present study was to evaluate the different features of such instruments and how they cause ocular fatigability. METHODS: Under the same settings and circumstances, 75 participants were asked to read the novel, "You without me?" in different formats including paper book, E-book (biscuit(R), Interpark Inc., LG INNOTECK LTD, Korea) and LCD reader (iPad(R), Apple Inc., United States). For every 10 minutes of reading, 10 minutes of rest was provided. After finishing the novel, the participants were asked to complete a questionnaire, and the data obtained were statistically analyzed using univariate analysis. RESULTS: Compared to paper book readers (1.93), LCD readers (2.40) complained of insufficient lubrication in the eye (p = 0.038), and experience more letter-floating illusions (1.92) than did paper book readers (1.49) (p = 0.043). Moreover, compared to the other formats including the E-book (20%) and paper book (8%), LCD readers (72%) experienced more significant glare symptoms (n = 23) and could read at a faster rate than the other groups of readers (n = 26). CONCLUSIONS: In the present study, the I-Pad caused ocular fatigability more easily than the other formats examined. No statistical significance of ocular discomfort or fatigability was observed among the different format groups. Further investigation with a greater number of age-controlled participants should be conducted prior to designing a study to determine the best format for reading.
Dry Eye Syndromes ; Eye ; Glare ; Illusions ; Lubrication ; Multimedia ; Surveys and Questionnaires

Diagnosing pseudoaneurysms of the popliteal artery is usually straightforward in physical examinations and imaging findings. However, when a pseudoaneurysm shows a soft tissue mass with adjacent osseous change, it can mimic a bone tumor or a soft tissue sarcoma. We present a case of a 65-year-old man who had a pseudoaneurysm of the popliteal artery showing soft tissue mass and insinuating into the intramedullary cavity of the tibia. This presented case emphasizes the importance of considering pseudoaneurysms in the differential diagnosis of an apparent soft tissue mass with pressure erosion in adjacent bone.
Aged ; Aneurysm, False ; Diagnosis, Differential ; Humans ; Hydrazines ; Physical Examination ; Popliteal Artery ; Sarcoma ; Tibia

BACKGROUND: Sternal wound infection (SWI) is an important complication after cardiac surgery. The aim of this study was to investigate the predictors affecting sternal wound infection and preventive factors including short term Vancomycin therapy in patients who underwent coronary artery bypass grafting (CABG). MATERIAL AND METHOD: A retrospective study was done using data collected from January 2001 through December 2007. This included 219 patients who had isolated CABG. The definition of SWI was documentation from a microbiological study and a requirement for simple closure or other surgical revision. RESULT: The overall incidence of SWI was 7.8% (n=17). The causative organisms were methicillin resistant Staphylococcus aureus (MRSA, n=13), methicillin resistant Staphylococcus epidermidis (n=2), Pseudomonas aeruginosa (n=1), and Candida albicans (n=1). Ten cases had deep sternal wound infection with mediastinitis; 7 cases had a superficial wound infection. Infection-related mortality was low (1/17; 6%). Diabetes mellitus (p=0.006) and smoking history (p=0.020) were factors that predicted high risk. Short term use of vancomycin decreased the incidence of MRSA-associated SWI (p=0.009). For treatment, curettage and rewiring or flap were needed in most cases (88%, n=14). CONCLUSION: Patients who had diabetes mellitus and a smoking history need careful management. Short term use of vancomycin is effective for prevention of SWI with MRSA.
Candida albicans ; Coronary Artery Bypass ; Coronary Vessels ; Curettage ; Diabetes Mellitus ; Humans ; Incidence ; Methicillin Resistance ; Methicillin-Resistant Staphylococcus aureus ; Pseudomonas aeruginosa ; Reoperation ; Retrospective Studies ; Risk Factors ; Smoke ; Smoking ; Staphylococcus aureus ; Staphylococcus epidermidis ; Sternum ; Thoracic Surgery ; Vancomycin ; Wound Infection

OBJECTIVE: We analyzed quantification of mitochondria DNA (mtDNA) to investigate the relationship of mitochondria and pathogenesis of PCOS. MATERIALS AND METHODS: Peripheral blood samples were collected from 28 patients with PCOS who were under the inclusion criteria for PCOS and from 28 healthy controls. Genomic DNA was used to analyze real-time PCR for mtDNA copy number quantification. The mtDNA copy number was compared between the control and PCOS groups. All data was expressed as mean +/- SD. Statistical analysis was assessed by t-test. RESULTS: In this study, the mtDNA CT was 11.67+/-0.422 in PCOS patients and 11.51+/-0.722 in control group, respectively. The mtDNA copy number was 1726410.71+/-407858.591 the patients of in PCOS and 2167887.51+/-252459.28 in control group (p=0.08), respectively. CONCLUSION: In our study, using real-time PCR, there was a tendency of lower mtDNA copy number in the patients of PCOS when comparing to the control group even though statistical difference was not significant. However, more extensive analysis is required to clarity relationship between mtDNA copy number and pathogenesis of PCOS.
DNA ; DNA, Mitochondrial* ; Humans ; Mitochondria ; Polycystic Ovary Syndrome* ; Real-Time Polymerase Chain Reaction

There are two pathogenic models of renovascular hypertension (RVH) originating from renal artery stenosis. We noted a case of a boy who had severe hypertension with atrophic left kidney, hypertrophic right kidney, a segmental stricture of the abdominal aorta, and total occlusion of the right renal artery. Due to the atrophic change of the contralateral, unclipped left kidney, this patient presented with various clinical manifestations related to both models of pathogenesis of RVH occurring at the same time. We conclude that this patient presented with the middle stage of the two RVH pathogenetic models, so called the 'one-clip, one and half-kidney model.'
Aorta, Abdominal ; Child* ; Constriction, Pathologic ; Humans ; Hypertension ; Hypertension, Renovascular* ; Kidney ; Male ; Renal Artery ; Renal Artery Obstruction ; Renin-Angiotensin System ; Takayasu Arteritis*