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

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

BACKGROUND: The purpose of this study is to compare the effectiveness of thoracic epidural anesthesia (TEA) and general anesthesia (GA) in terms of side effects, postoperative pain control and the cost of anesthesia for breast mass excision surgery. METHODS: Forty-three patients rated as ASA physical status class 1 or 2 who underwent breast mass excision surgery were included in the study. In the TEA group (n = 20), 2% lidocaine (3 ml) and 0.5% bupivacaine (3 ml) were administered via the epidural route. Blood pressure and heart rate were measured before and at 3, 6, 9, 12, 15, 18 21, 24, 27 and 30 minutes after epidural injection. In the GA group (n = 23) patients were maintained by general anesthesia with enflurane and 50% nitrous oxide in oxygen. After the operation, patients were given NSAID-diclofenac sodium as they wanted. Analgesic requirement, satisfaction score, and anesthesia-related side effects were recorded 1 day after surgery. Satisfaction scores of the surgeons and patients were recorded as excellent (4 point), good (3 point), fair (2 point), and unacceptable (1 point). RESULTS: Overall satisfaction scores and side effects were not significantly different between the TEA group and the GA group. Anesthesia cost (80,883.2 +/- 3956.9 vs 32,284.8 +/- 1209.4 won) were significantly lower in the TEA group than in the GA group. CONCLUSIONS: There were not significant differences in satisfaction scores and postoperative side effests between the TEA and GA groups. TEA provided lower anesthesia cost than GA for breast mass excision surgery.
Anesthesia ; Anesthesia, Epidural* ; Anesthesia, General* ; Blood Pressure ; Breast* ; Bupivacaine ; Enflurane ; Heart Rate ; Humans ; Injections, Epidural ; Lidocaine ; Nitrous Oxide ; Oxygen ; Pain, Postoperative ; Sodium ; Tea

No abstract available.

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