Epstein's syndrome is a rare disease whish is characterized by the association of thrombocytopenia, macrothrombocytopathia, nephritis and deafness. We experienced a case of Epstein's syndrome in a 12 years old male patient who was presented with a life long history of bleeding, usually as epistaxis, bilateral sensorineural deafness and hematuria with proteinuria starting in late childhood. Hematologic studies showed thrombocytopenia with giant platelets and anemia. A bone marrow aspirate revealed the megakaryocytes to be adequate in number and many giant size platelets. Platelet do not respond to addition of A and epinephrine; collagen and ristocetin induced agglutination response is decreased. It is difficult to be certain the association of thrombocytopenia with giant platelets, nephritis and deafness constitutes a new hereditary disease with a distinct pathogenesis or if it is an expansion of the well recognized Alport's syndrome of hereditary nephritis deafness. We report a case of Epstein's syndrome syndrome with brief review of related literatures.
Agglutination ; Anemia ; Blood Platelets ; Bone Marrow ; Child ; Collagen ; Deafness ; Epinephrine ; Epistaxis ; Genetic Diseases, Inborn ; Hematuria ; Hemorrhage ; Humans ; Male ; Megakaryocytes ; Nephritis ; Nephritis, Hereditary ; Proteinuria ; Rare Diseases ; Ristocetin ; Thrombocytopenia

No abstract available.
Parenteral Nutrition, Total

We report a patient with an isolated cerebellar nodulus infarction who presented with periodic alternating nystagmus (PAN), perverted head-shaking nystagmus (pHSN), and loss of tilt suppression of the vestibulo-ocular reflex (VOR). Several days after the initial symptoms resolved, the patient also presented with purely vestibular syndrome with vertigo, spontaneous horizontal nystagmus without the usual signs of cerebellar dysfunction. The chain of those symptoms were ascribed to ischemia of the cerebellar nodulus.
Cerebellar Diseases ; Humans ; Infarction* ; Ischemia ; Nystagmus, Pathologic* ; Reflex, Vestibulo-Ocular ; Vertigo

PURPOSE: Development of reconstruction plastic surgery for burn treatment and application SRNT (skin rehabilitation nursing therapy) have promoted improvement of dysmorphic feature and functional recovery. In case of deeper and extensive burn, however, treatment of burn scar after plastic surgery and SRNT may be unsatisfactory to burn patients. Especially burn patients having dysmorphic feature on exposed area like face suffer from psychological distress like depression, anxiety, guiltyfeeling, feeling of worthlessness, psychicinertia. Rehabili-Medical Makeup have performed congenital patient having hemangioma and vitiligo using covermark, and this is one of three technical effect of specialized skin rehabilitation nurses. Application of Rehabili-Medical Makeup covers burn scar afraid of exposure and resolves psychological distress, which makes burn patients gain confidence in themselves and helps their return to society. METHODS: Subjects of one's study are 10 burn patients that received SRNT from january 2007 to january 2011 and wanted Rehabili-Medical Makeup. We performed Rehabili-Medical Makeup using covermark foundation. practiced stress measuring test (MEDICORE SA-3000P, NIPRO Cocoro Stress Meter(R)) measured satisfaction (skin color, texture, duration, effectiveness and participation in education) using questionnaire. RESULTS: In stress measureing test after Rehabili-Medical Makeup patients maintained physiological balance. Satisfaction questionnaire show that patients are satisfied with every item. CONCLUSION: Rehabili-Medical Makeup resolves psychological distress and helps their return to society, which is final goal of rehabilitation. Hereafter we suggest to apply and expand education for managing patient's burn scar for themselves.
Anxiety ; Burns ; Cicatrix ; Depression ; Hemangioma ; Humans ; Surveys and Questionnaires ; Rehabilitation Nursing ; Skin ; Surgery, Plastic ; Vitiligo

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BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) involves dysregulation of the complement system, but whether this also occurs in thrombotic thrombocytopenic purpura (TTP) remains unclear. Although these conditions are difficult to differentiate clinically, TTP can be distinguished by low (<10%) ADAMTS13 activity. The aim was to identify the differences in complement activation products between TTP and aHUS and investigate ADAMTS13 activity as a prognostic factor in aHUS. METHODS: We analyzed patients with thrombotic microangiopathy diagnosed as TTP (N=48) or aHUS (N=50), selected from a Korean registry (N=551). Complement activation products in the plasma samples collected from the patients prior to treatment and in 40 healthy controls were measured by ELISA. RESULTS: The levels of generalized (C3a), alternate (factor Bb), and terminal (C5a and C5b-9) markers were significantly higher (all P<0.01) in the patients than in the healthy controls. Only the factor Bb levels significantly differed (P=0.008) between the two disease groups. In aHUS patients, high normal ADAMTS13 activity (≥77%) was associated with improved treatment response (OR, 6.769; 95% CI, 1.605–28.542; P=0.005), remission (OR, 6.000; 95% CI, 1.693–21.262; P=0.004), exacerbation (OR, 0.242; 95% CI, 0.064–0.916; P=0.031), and disease-associated mortality rates (OR, 0.155; 95% CI, 0.029–0.813; P=0.017). CONCLUSION: These data suggest that complement biomarkers, except factor Bb, are similarly activated in TTP and aHUS patients, and ADAMTS13 activity can predict the treatment response and outcome in aHUS patients.
Atypical Hemolytic Uremic Syndrome ; Biomarkers ; Complement Activation ; Complement System Proteins ; Enzyme-Linked Immunosorbent Assay ; Humans ; Mortality ; Plasma ; Purpura, Thrombotic Thrombocytopenic ; Thrombotic Microangiopathies

PURPOSE: The aim of this study was to identify the relationship between urinary incontinence (UI) and low back pain (LBP) discomfort and disability, static balance, and demographic factors. METHODS: A total of 348 women aged 20-80 years were included in this cross-sectional study. The general characteristics of the subjects and the main outcome (UI condition, LBP discomfort, LBP disability, and static balance ability) were assessed by using clinical questionnaires and assessment tools. RESULTS: Of all the subjects, 22.8% had experienced UI. Women with UI showed a significantly higher relationship of LBP and disability, and static balance ability (P<0.01). We found a significant correlation between UI, age, LBP and disability, and static balance ability (P<0.01). CONCLUSIONS: These findings suggest that UI correlates negatively with LBP discomfort, LBP disability, and static balance ability. Further studies should focus on the identification of the precise mechanisms underlying UI and its related physical symptoms and on the development of therapeutic strategies to manage this condition.
Aged ; Cross-Sectional Studies ; Female ; Humans ; Low Back Pain ; Urinary Incontinence

PURPOSE: This study aimed to improve prostate biopsy compliance by analyzing the factors that influence the acceptance of prostate biopsy by patients to whom transrectal ultrasound (TRUS)-guided prostate biopsy is recommended for suspected prostate cancer. MATERIALS AND METHODS: The subjects of this study were 268 patients to whom TRUS-guided prostate biopsy was recommended from January to June 2011 and who completed a questionnaire. Patients who showed a prostate-specific antigen (PSA) increase to more than 4.0 ng/mL or abnormal findings on a digital rectal examination and TRUS were recommended to undergo prostate biopsy. The questionnaire consisted of 9 questions about the subjects' demographic characteristics and 15 questions that assessed their knowledge of prostate disease. Fisher exact probability test was conducted to assess the influence of the demographic characteristics and levels of knowledge of prostate disease on acceptance of prostate biopsy. RESULTS: The mean age of the subjects was 66.2 years (range, 43-83 years). Of the cohort, 188 patients (70.7%) agreed to the prostate biopsy and 78 patients (29.3%) refused. In terms of demographic characteristics, the patients' acceptance of prostate biopsy was associated only with education level. Patients with relatively lower education levels had a higher acceptance rate for prostate biopsy (80.0% vs. 65.9%, p=0.018). Other demographic factors, as well as the degree of knowledge of prostate disease, had no significant effect on the acceptance rate. CONCLUSIONS: The patients' acceptance of prostate biopsy can be influenced by demographic characteristics, especially education level. Therefore, when prostate biopsy is recommended to patients, their demographic characteristics should be taken into consideration.
Adult ; Aged ; Aged, 80 and over ; Biopsy, Needle/methods/psychology ; Health Knowledge, Attitudes, Practice ; Humans ; Kallikreins/blood ; Male ; Middle Aged ; *Patient Acceptance of Health Care ; Prospective Studies ; Prostate-Specific Antigen/blood ; Prostatic Neoplasms/*pathology/psychology/ultrasonography ; Republic of Korea ; Socioeconomic Factors ; Ultrasonography, Interventional

PURPOSE: We investigated the therapeutic effects of monoclonal anti-TNF antibody (infliximab) on experimental uveitis. METHODS: Twenty New Zealand White rabbits were immunized with Mycobacterium tuberculosis H37Ra antigen and then challenged with intravitreal injection of tuberculin antigen to introduce a uveitis. Then infliximab was injected into rabbit eyes at an intravenous concentration of 5 mg/kg and intravitreal concentrations of 1 mg/0.1mL and 100 microg/0.1mL. As a control, the vehicle was injected intravenously or intravitreally. To evaluate the therapeutic effects, inflammation was assessed by slit lamp biomicroscopy and scored according to the severity of inflammation. The animals were also evaluated by electroretinography and histopathology. RESULTS: Regardless of the administration route, inflammatory activities of anterior chamber and engorgement of vascular structures were reduced in the infliximab treated group compared to control. Different administration routes and different concentrations of infliximab did not affect the therapeutic outcome of the clinical scoring. Intravenous (5 mg/kg) and intravitreal diluted (100 microg/0.1mL) infliximab injection groups showed significant improvement in electroretinographic findings and significant reduction of inflammatory cells with preservation of retinal tissue architecture on histopathologic examination. However, focal loss of the photoreceptor outer segment is observed in intravitreal undiluted (1 mg/0.1 mL) infliximab injected eyes. CONCLUSIONS: Infliximab may be a useful treatment modality to suppress ocular inflammation in experimental uveitis models in rabbits.
Animals ; Anterior Chamber ; Electroretinography ; Inflammation ; Intravitreal Injections ; Mycobacterium tuberculosis ; Necrosis* ; Rabbits ; Retinaldehyde ; Tuberculin ; Tumor Necrosis Factor-alpha ; Uveitis* ; Infliximab

The efficacy of hydrocolloid occlusive dressing technique was compared with that of the conventional wet-to-dry gauze dressing technique in decubitus ulcer of stage I and II. Forty-four patients were randomly divided into two treatment groups and each received treatment according to the two different protocols. As a result, 80.8% of the hydrocolloid occlusive dressing group (group 1) and 77.8% of the conventional wet-to-dry gauze dressing group (group 2) healed completely with no statistically significant difference between the two groups. However, the time required for complete healing was shorter in group 1 with 18.9 days compared to 24.3 days in group 2. Ulcer healing speed was also slightly faster in group 1 with 9.1 mm2/day compared to 7.9 mm2/day for group 2. Average treatment time spent by a medical staff member was significantly shorter in group 1 with 20.4 minutes/day compared to 2017 minutes/day in group 2. The hospital cost of the ulcer treatment was higher in group 2 compared to group 1 even without taking into consideration the medical personnel's labor cost. These results indicate that the hydrocolloid occlusive dressing technique offers less time consuming and less expensive method of treatment compared to the conventional technique in stage I andII decubitus ulcers.
Adult ; Aged ; Comparative Study ; Decubitus Ulcer/*therapy ; Female ; Human ; Male ; Middle Age ; *Occlusive Dressings