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

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

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

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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

BACKGROUND: Obesity is not anly one of the risk factors that affects the prevalence and aggravation of chronic diseases, but is also the cause of social unfitness because slimness has become a standard of beauty. We surveyed on the satisfaction about self body image, the factors which are related to it and weight control behavior of the adolescents in a local area of Korea. METHODS: The survey was performed in a high school of Pocheon on August, 1996. Five classes out of 14 classes in 1st and 2nd grade were selected. The body mass index(BMI) was calculated from the measured height and weight. The satisfaction about body image which was estimated by the difference between present body image and wanted body image in 9 graded figures of body image was obtained from the questionnaire. The information about sex, religion, history of being teased about body image, body image of their parents, mean duration of watching TV in a day, body esteem, and binge-eating was obtained from the questiannaire and we evaluated the relationship between all of the above with BMI and body image satisfaction. We also investigated on the interest in education about weight control and efforts made by adolescents from the questionnaire on weight control behavior. RESULTS: Total of 224 students participated in this study and only 14.7% of them were satisfied with their body image. 58.9% wanted weight reduction and 26.4% wanted weight gain. They were more dissatisfied with their body image when they were girls(p<0.01), had the experience of being teased about their body image(p<0.01), had low body esteem(p<0.01), and had high BMl(p<0.01). We could not find statistically significant relationship between body image satisfaction and other factors such as religion, the duration of watching TV in a day, the body image of their parents and binge eating. 64.3% of the subjects tried to control their weight and most(87.5%) tried to lose weight. The selected methods were diet control(65.3%) and exercise(52.1%) and the most preferred method of diet control was skipping meah(45.7%). CONCLUSIONS: Most of adolescents were not satisfied with their body image and this dissatisfaction was related to gender, history of being teased about their body image, BMI, and body esteem. Many of them had tried to lose weight and among the methods of weight reduction, there were behaviors that were injurous to their health and growth
Adolescent* ; Beauty ; Body Image* ; Bulimia ; Chronic Disease ; Diet ; Education ; Humans ; Korea ; Obesity ; Parents ; Prevalence ; Risk Factors ; Weight Gain ; Weight Loss ; Surveys and Questionnaires

Since Wands et al. reported for the first time in 1975 the reactivation of the hepatitis B virus in hematologic disease patients who had been receiving chemotherapy, the efficacy of chemotherapy and immunosuppressants has improved. As a result, the frequency of the reactivation of hepatitis B is increasing. Reported herein is a case of a non-Hodgkin lymphoma patient in her 70s who was suspected to have had HBsAg negative/anti-HBs negative occult HBV infection. The patient experienced fulminant hepatitis caused by the reactivation of hepatitis B, and died three months after the R-CHOP regimen was completed. In the HBsAg negative plus HBV DNA-negative case, there were few instances of viral activation of HBV. In this case, antiviral therapy was needed when the patient was confirmed to have become HBV DNA positive through regular monitoring, but its necessity is often overlooked, unlike the preemptive antiviral treatment in the HBsAg positive cases.
DNA ; Drug Therapy* ; Hematologic Diseases ; Hepatitis B Surface Antigens ; Hepatitis B virus ; Hepatitis B* ; Hepatitis* ; Humans ; Immunosuppression ; Immunosuppressive Agents ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Virus Activation ; Rituximab

Adventitial cystic disease of the popliteal artery is a rare disorder that causes localized stenosis or occlusion by compression of the vessel lumen. The disease produces lower extremity claudication, typically in young and middle-aged men. We report a case of the adventitial cystic disease of the popliteal artery in a 55-year-old man with symptom of left calf claudication. Diagnosis was done by ultrasound, angiography, and magnetic resonance imaging. The patient was treated with resection of the diseased popliteal artery followed by interposition of saphenous vein graft. Postoperatively, the symptom and sign resolved completely.
Angiography ; Constriction, Pathologic ; Diagnosis ; Humans ; Lower Extremity ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Popliteal Artery* ; Saphenous Vein ; Transplants ; Ultrasonography

No abstract available.
Epilepsy ; Humans ; Seizures ; Ventricular Fibrillation