Apert syndrome is an uncommon, congenital disorder characterised by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. The original description was presented by Troquart in 1886, and acrocephaloyndactyly was named by Apert in 1906. Since then, more than 200 cases have been reported in the world upto 1970. Recently, we have experienced three for typical Apert syndrome and made a brief related literature review
Acrocephalosyndactylia* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Craniosynostoses ; Foot ; Hand ; Head ; Skull ; Syndactyly

No abstract available.
Humans ; Infant, Newborn* ; Streptococcus pneumoniae* ; Streptococcus*

These days, the computer network has become the most popular and widely used source of information. There are two kinds of computer networks, the first one is multimedia internet world wide web site and the second one is text based commercial network (etc. Hitel, Chollian. Nownuri, Unitel). With the rapid development of internet, various kinds of medical-related sites are newly appearing on the internet. Even in Korea, several medical-related information sites are on service, however, there are very few compared with foreign sites. World wide web is popular, and creating the homepage for each hospital is becoming more important. In Korea, the number of homepage of about medicine and medical institutes, is increasing. For the construction of Soonchunhyang University Hospital's Computer Information Network, the authors first investigate Korean medical-related information sites available in internet and commercial network. After researching the authors then construct the Computer Information Network of Soonchunhyang University Hospital. This article is written with the intent of assisting those interested in creating and managing their own computer information network system by means of providing relevant data obtained during this process.
Academies and Institutes ; Information Services* ; Internet ; Korea ; Multimedia

No abstract available.
Hirschsprung Disease* ; Hypoventilation*

We report on a patient with spontaneous rupture of hemorrhagic renal cyst into the pyelocaliceal system. He had no history of trauma, bleeding disorders, urinary tract infection or urinary tract obstruction. Gross hematuria followed by left flank pain occurred 1 day prior to admission. Although radiologic findings were suggestive of rupture of renal cyst into the calyx or calyceal diverticulum. exclusion of malignant disease or renal carbuncle was difficult. He was managed by partial nephrectomy. Microscopic findings revealed ruptured cyst lined with low cuboidal epithelium without evidence of malignancy.
Carbuncle ; Diverticulum ; Epithelium ; Flank Pain ; Hematuria ; Hemorrhage* ; Humans ; Nephrectomy ; Rupture ; Rupture, Spontaneous* ; Urinary Tract ; Urinary Tract Infections

PURPOSE: Cepharanthin (R) has been reported to improve the symptoms of intractable or steroid-resistant chronic idiopathic thrombocytopenic purpura (ITP). We report the clinical efficacy of oral high dose Cepharanthin (R) in refractory chronic ITP. METHODS: We analyzed eleven patients who were diagnosed as chronic ITP at the Department of Pediatrics, Seoul National University Hospital from January, 2002 to February, 2004. After informed consent patients with platelet count below 20, 000/mm3 were treated with Cepharanthin (R) at the initial dose of 5 gm/day. The doses were increased up to 15 gm/day if there was no response. For the purposes of this study, complete remission was defined as an elevation of the platelet count > or =50, 000/mm3 from the baseline after 4 weeks of treatment. Partial remission was defined as an elevation of the platelet count 20, 000/mm3 and < = or50, 000/mm3 from the baseline. RESULTS: Seven boys and four girls with a median age of 10 were enrolled. Two to four weeks after the initiation of this therapy, 4 patients showed their platelet counts over 50, 000/mm3 1 patients reached partial remission state, 6 patients had no response. Side effects of Cepharanthin (R) were not observed in all patients. CONCLUSION: We suggest that the oral administration of Cepharanthin (R) could be a beneficial and a safe treatment strategy for the refractory chronic ITP patients. Further studies should be performed to elucidate the mechanism of responses.
Administration, Oral ; Child* ; Female ; Humans ; Informed Consent ; Pediatrics ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic* ; Seoul

No abstract available.
Down Syndrome* ; Leukemia*

We report a case of SLE with antiphospholipid antibodies presented initially with severe bleeding. A six-year-old boy was admitted due to severe nasal bleeding for 2 months. The boy showed typical malar rash. The laboratory tests indicated that his platelet count was 80,000/mm3 and the PT and the aPTT were markedly prolonged. A number of clotting factors were decreased, including factorsll<12%, Vll: 42%, lX : 38%, Xl: 41%, and Xll: 16%. Urinalysis showed hematuria and proteinuria, and 24-hour urine protein was 1.37g/day. Venereal Disease Research Laboratory (VDRL) test was false positive, Coombs test, lupus anticoagulants and anticardiolipin antibodies (IgG and IgM) were positive. His symptoms and laboratory tests fulfilled the criteria of SLE with antiphospholipid antibody. Renal pathology showed lupus nepritis (diffuse proliferative glomerulonephritis, class lV). After steroid therapy, his nasal bleeding stopped immediately, and laboratory findings became normalized. This case showed the tendency of paradoxic bleeding, instead of the expected thrombosis which can be found in this type of patient. We anticipate it is mainly due to pronounced prothrombin deficiency.
Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid* ; Anticoagulants ; Coombs Test ; Epistaxis ; Exanthema ; Glomerulonephritis ; Hematuria ; Hemorrhage* ; Humans ; Hypoprothrombinemias ; Lupus Erythematosus, Systemic* ; Male ; Pathology ; Platelet Count ; Proteinuria ; Sexually Transmitted Diseases ; Thrombosis ; Urinalysis

We report a case of SLE with antiphospholipid antibodies presented initially with severe bleeding. A six-year-old boy was admitted due to severe nasal bleeding for 2 months. The boy showed typical malar rash. The laboratory tests indicated that his platelet count was 80,000/mm3 and the PT and the aPTT were markedly prolonged. A number of clotting factors were decreased, including factorsll<12%, Vll: 42%, lX : 38%, Xl: 41%, and Xll: 16%. Urinalysis showed hematuria and proteinuria, and 24-hour urine protein was 1.37g/day. Venereal Disease Research Laboratory (VDRL) test was false positive, Coombs test, lupus anticoagulants and anticardiolipin antibodies (IgG and IgM) were positive. His symptoms and laboratory tests fulfilled the criteria of SLE with antiphospholipid antibody. Renal pathology showed lupus nepritis (diffuse proliferative glomerulonephritis, class lV). After steroid therapy, his nasal bleeding stopped immediately, and laboratory findings became normalized. This case showed the tendency of paradoxic bleeding, instead of the expected thrombosis which can be found in this type of patient. We anticipate it is mainly due to pronounced prothrombin deficiency.
Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid* ; Anticoagulants ; Coombs Test ; Epistaxis ; Exanthema ; Glomerulonephritis ; Hematuria ; Hemorrhage* ; Humans ; Hypoprothrombinemias ; Lupus Erythematosus, Systemic* ; Male ; Pathology ; Platelet Count ; Proteinuria ; Sexually Transmitted Diseases ; Thrombosis ; Urinalysis

We analyzed the clinical and laboratory features of ten children with acute megakaryoblastic lukemia (M7)and compared the findings with those reported in the literature. The diagnosis was supprted by ultrastructural examination of platelet peroxidase or immunophenotyping for glycoprotein IIb/IIIa. Of the ten children, five were girls and five were boys. The median age at diagnosis was 13 months. Two patients had prominent myelofibrosis and one patient had Down syndrome. Nine patients were treatd with low-dose cytosine arabinoside (10mg/m2)administered intravenously, or subcutaneously, or intramuscularly, twice daily in 21 day courses. Seven patients achieved hematologic response and three patients are alive without evidence of disease. The 4 year event free survival rate was30.0%. It is our impression that the prevalence of acute megakaryoblastic leukemia has been under-estimated, and low-dose cytosine arabinoside treatment may be of value in its management. This approach may be particularily useful in hospitals with scarce well-equipped facilities, since this protocol does not induce profound marrow hypoplasia and intensive supportive measures are not required as they would be with the use of more aggressive drug combination.
Blood Platelets ; Bone Marrow ; Child* ; Cytarabine ; Diagnosis ; Disease-Free Survival ; Down Syndrome ; Female ; Glycoproteins ; Humans ; Immunophenotyping ; Leukemia, Megakaryoblastic, Acute* ; Megakaryocyte Progenitor Cells ; Peroxidase ; Prevalence ; Primary Myelofibrosis