No abstract available.
Ichthyosiform Erythroderma, Congenital*

Reactive perforating collagenosis(RPC) is a kind of perforating dermatosis characterized by transepidermal elimination of altered dermal collagen. RPC is classified into two forms; childhood or inherited form, and adulthood or acquired form. Acquired RPC is reported to occur in association with the severe complicated diabetes mellitus, chronic renal failure and other diseases. We describe a 43-year-old Korean woman with atypical acquired RPC associated with uncontrolled diabetes mellitus and severe pruritus. The histopathologic findings of the lesions showed neither transepidermal channel nor cup-shaped epidermal depression. Multiple degenerated collagen bundles arranged vertically and were eliminated through epidermis to the surface individually. Transmission electron microscopic findings showed the same as typical RPC. Skin lesions improved after the insulin subcutaneous injections, UVB phototherapy and antihistamine administration.
Adult ; Collagen ; Depression ; Diabetes Mellitus ; Epidermis ; Female ; Humans ; Injections, Subcutaneous ; Insulin ; Kidney Failure, Chronic ; Phototherapy ; Pruritus ; Skin ; Skin Diseases

Spindle cell hemangioendothelioma was first described in 1986 to characterize a particular angiomatous lesion which shows different clinical features, however, it has recently been designated as spindle cell hemangioma (SCH) due to its benign nature. Microscopically, this peculiar vascular tumor consists of cavernous blood vessels intermixed with solid areas predominantly composed of spindle cells, resembling Kaposi sarcoma. A 39-year-old woman presented with a bean-sized, tender, bluish nodule on the pulp of her right thumb. The 1 year old lesion had increased slowly in size over this time. Histologic examination of the lesion showed that it was composed of large irregularly-dilated, thin-walled cavernous blood spaces containing organizing thrombi, numerous red blood cells, and a spindle cell mass which was partially connected with the vessel wall and invaded the stroma. A diagnosis of SCH was made based on histologic and immunohistochemical findings. After the lesion was totally excised, there has been neither recurrence nor occurence of new lesions to date.
Adult ; Blood Vessels ; Diagnosis ; Erythrocytes ; Female ; Hemangioendothelioma ; Hemangioma* ; Humans ; Recurrence ; Sarcoma, Kaposi ; Thumb

Nodular fasciitis represents reactive fibroblastic or myofibroblastic proliferative lesions which may be misdiagnosed as sarcomas due to the rich cellularity, mitotic activity and variant morphologic pattern. It arises in the subcutaneous tissue, skeletal muscles, and fascia. Uncommon clinical and pathological variants of nodular fasciitis, such as intradermal, intravascular, cranial, ossifying, parosteal and proliferative fasciitis, have been described. We present a 23-year-old man with a one year history of a 2x1.5cm sized hard tender nodule on the scalp which is partially depressed at the margin. Histological examination revealed clusters of spindle cells in a myxoid background, chronic inflammatory cells and extravasated red cells in the dermis. Immunohistochemically, the spindle cells showed positivity of vimentin or smooth muscle actin, and negativity of desmin or S-100 protein. The staining results support its myofibroblastic origin. There is no involved bony lesion on the brain computed tomography (CT). Therefore we report a rare case of intradermal fasciitis arising on the scalp.
Male ; Humans

Hemodialysis is a major mode of renal replacement therapy in children as well as CAPD. PermCath , a dual lumen silicone atrial catheter with a dacron cuff, has been used as vascular access for hemodialysis. Between Aug. 1, 1994 and Mar. 31, 1996, 29 children had been received PermCath catheter implantation for hemodialysis in the Department of Pediatrics, Seoul National University Children's Hospital. Their medical records were analyzed retrospectively. The mean age and weight of the patients were 9.8+/-4.6years (6months-17years) and 25.7+/-11.0kg (6.2kg-40kg), respectively. For short-term use (60days) users, mean life span of the catheter was 230.8 +/-158.6days (61-591days) and the actuarial catheter survival rate at 1 year was 44 %. The mean recirculation rate checked in 5 patients was 9.6+/-4.0%. The rate of thrombotic occlusion was 1.5/1,000 catheter days. Most of the clots except one could be lysed with local urokinase therapy. The rate of catheter associated fever was 0.6/1,000 catheter days. One catheter needed revision due to malposition and local bleeding developed during the procedure. PermCath catheterization was immediate, durable and relatively safe vascular access in childhood hemodialysis.
Arteriovenous Fistula ; Catheterization* ; Catheters* ; Child ; Fever ; Hemorrhage ; Humans ; Kidney Transplantation ; Medical Records ; Pediatrics ; Peritoneal Dialysis, Continuous Ambulatory ; Polyethylene Terephthalates ; Renal Dialysis* ; Renal Replacement Therapy ; Retrospective Studies ; Seoul ; Silicones ; Survival Rate ; Thrombosis ; Urokinase-Type Plasminogen Activator

Primary peritonitis usually refers to a bacterial infection of the peritoneal cavity without a demonstrable intra-abdominal source. Most cases occur in children with ascites resulting from nephrotic syndrome or cirrhosis. Rarely, it may occur in previously healthy children less than 7 years of age, usually a girl. Distinguishing primary peritonitis from appendicitis may be impossible in patients without a history of nephrotic syndrome or cirrhosis. Accordingly, the diagnosis of primary peritonitis is made only at laparotomy. We report one case of primary pneumococcal peritonitis in a 27-month-old female who underwent explorative laparotomy to discover the cause of suspicious intestinal perforation and mechanical ileus. Later, pneumococci were cultured in blood and gram-positive diplococci were isolated from the pus of peritoneal cavity.
Appendicitis ; Ascites ; Bacterial Infections ; Child* ; Child, Preschool ; Diagnosis ; Female ; Fibrosis ; Humans ; Ileus ; Intestinal Perforation ; Laparotomy ; Nephrotic Syndrome ; Peritoneal Cavity ; Peritonitis* ; Suppuration

Mixed tumor of the skin, so called chondroid syringoma, is a benign epithelial neoplasm with glandular or ductal differentiation surrounded by myxoid stroma. It is most frequently located on the head and neck and presents as an asymptomatic, firm, subcutaneous nodule. Mixed tumor with follicular, sebaceous and apocrine differentiation is considered as an expression of the common embryologic origin of elements of the folliculo-sebaceous-apocrine unit. We report a case of mixed tumor of the skin with follicular, sebaceous and apocrine differentiation.
Adenoma, Pleomorphic ; Head ; Neck ; Neoplasms, Glandular and Epithelial ; Skin

No abstract available.
Glomerular Filtration Rate*

PURPOSE: The aim of this study was to identify the predictive factors in the early laboratory findings for cardiac sequelae in Kawasaki disease(KD). METHODES: A retrospective review of the records was conducted of all children with KD who were admitted to the Ulsan Dongkang General Hospital, Masan Samsung Hospital, and Gyeongsang National University Hospital between January 1995 and December 1999. We analyzed and compared the early laboratory findings between the patients with and without coronary artery dilatation. RESULTS: A total of 981 patients were divided into two groups : 826 patients(84.3%) with normal coronary artery and 155 patients(15.7%) with coronary artery dilatation. Age and sex were not significantly different between the two groups. The mean serum C-reactive protein(CRP) in the coronary artery dilatation group and in the normal coronary artery group were 5.0 mg/dl(+/-5.3) and 4.1 mg/dl(+/-5.0), respectively, with a significant difference(P<0.05), whereas the other early laboratory findings had no difference between the groups. CONCLUSION: This study shows that the early serum CRP was higher in patients with KD who had coronary artery dilatation than in those with normal coronary artery. There may be a strong possibility of cardiac sequelae at a high level of serum CRP. However, the cut-off value of serum CRP could not be determined for the prediction of cardiac sequelae in patients with KD.
Child ; Coronary Vessels ; Dilatation ; Hospitals, General ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Retrospective Studies ; Staphylococcal Protein A* ; Ulsan

When a surgeon fails to save intracranial segement of the facial nerve and can not identify the proximal segment of the facial nerve due to severe adhesion or severe brain stem compression by the tumor, the interpositional graft has no place in helping this patient. Hypoglossal-facial nerve (XII-VII) direct side to end anastomosis is the effective facial reanimation technique used in such a case. This technique compensates for the drawbacks of classical XII-VII anastomosis, and the bothersome strong mass movement and hemiparalysis of the tongue. We recently experienced a case where XII-VII direct side-to-end anastomosis was applied. We report our case with literature review.
Brain Stem ; Facial Nerve ; Facial Paralysis ; Humans ; Hypoglossal Nerve ; Tongue ; Transplants