Neurofibromatosis is a disease which involves both neuroectodermal and mesodermal tissue, and is characterized by cafe-au-lait spot, multiple subcutaneous neurofibromas, elephantiasis neuromatosa, a positive family history, and specific dystrophic osseous changes such as scoliosis, penciling of ribs, vertebral scalloping, a paravertebral soft tissue tumor, and congenital pseudarthrosis. The classic type of scoliosis in neurofibromatosis was known as a sharp localized short curve that is often rapidly progressive and produces severe deformity with dystrophic changes, but another form with long gentle curve is reported. Since conservative treatment is usually unsuccessful, posterior fusion with or without Harrington instrumentation is the treatment of choice even in young age when the curve is progressive. This paper was aimed to review our experience with 11 patients having neurofibromatosis and scoliosis, who were treated with posterior fusion and Harrington instrumentation from Jan. 1971 to Dec. 1980, and the results were as follows: 1. The average age that spinal deformity was observed was 7.7 years old, but the average age at treatment was 14.4. 2. Cafe-au-lait spot was observed in all cases, subcutaneous nodule in 7 cases, local gigantism in 2 cases, and positive family history in 3 cases. 2 cases were combined with congenital spinal anormalies. 3. Specific pattern in spinal deformity was not significant. There were 7 short curves less than 5 vertebrae involved and 6 long curves more than 6 vertebrae involved. 4. There were 5 kyphosis which had more than 50°, and those were usually combined with severe scoliosis. 5. Preoperative average degree of scoliosis was 93.8, and the final correction was 41.2° (43.9%) with loss of correction 5.3°(5.7%) after 3.2 year follow-up in average. 6. It shouid be educated for early detection at home and school, and for the importance of early treatment for the scoliosis, to prevent rapid increase of scoliosis in neurofibromatosis.
Cafe-au-Lait Spots ; Congenital Abnormalities ; Elephantiasis ; Follow-Up Studies ; Gigantism ; Humans ; Kyphosis ; Mesoderm ; Neural Plate ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Pectinidae ; Pseudarthrosis ; Ribs ; Scoliosis ; Spine

No abstract available.
Foot* ; Free Tissue Flaps*

No abstract available.
Alcoholism* ; Hospitals, General* ; Humans ; Prevalence*

Many oncogenes and tumor supressor genes have been identified and studied in colorectal carcinoma. Among them, p53 is a tumor supressor gene and its mutation is frequently noted in human tumors. E-cadherin is a cell adhesion molecule and associated with tumor differentiation. CD44 is a cell surface glycoprotein that plays a role in cell migration and metastasis. nm23 is a gene known to lower metastatic potential of tumors and has been proposed to be a metastasis supressor gene. Tumor angiogenesis is required for the expansion of the primary tumor and metastasis and its degree is related to the potential of malignancy. We studied the expression of p53, E-cadherin, nm23, CD44 and tumor angiogenesis in 36 cases of colorectal adenocarcinomas. They were compared with previously known prognostic factors such as the stage, tumor size, depth of invasion, differentiation, presence of lymphatic or venous invasion, the lymph node and distant metastasis. The results were as follows. 1) The expression of p53 was not significantly associated with any prognostic factors. 2) The expression of E-cadherin was significantly associated with tumor differentiation. In the well differentiated adenocarcinomas, its expression was higher than in the poorly differentiated adenocarcinoma. 3) The expression of nm23 was also significantly associated with tumor differentiation. In carcinoma with lymph node metastasis, the expression of nm23 was reduced, but statistically it was not significant. 4) The expression of CD44 was higher in tumors with lymph node metastasis than in tumors without lymph node metastasis, but it was not statistically significant. 5) The degree of microvessel density was significantly associated with lymphatic invasion. According to the above results, the expression of E-cadherin and nm23 are related to the differentiation of the tumor and tumor angiogenesis is related to the lymphatic invasion of the colorectal adenocarcinoma.
Adenocarcinoma* ; Cadherins* ; Cell Adhesion ; Cell Movement ; Colorectal Neoplasms ; Genes, vif ; Humans ; Lymph Nodes ; Membrane Glycoproteins ; Microvessels ; Neoplasm Metastasis ; Oncogenes ; von Willebrand Factor

No abstract available.

The ischial weight-bearing functional long leg brace is one of the new immobilization devices, which allows patients to walk using less energy than was required by other devices-e.g. cast, cast-brace, conventional long leg brace. It has advantages as minimal pain and irritation, more natural gait, convenience of free joint motion, and good skin hygiene. The brace consists of quadrilateral total contact thigh shell, bilateral up-rights, knee joint, tibial cuff band and ankle-foot section. The brace was applied to 10 patients with variable diseases treated at the Department of Orthopedic Surgery, Seoul National University Hospital, from Mar-74 to May-76. It was applied postoperatively to 4 cases of femoral shaft fractures and 1 case of fibrous dysplasia of the tibia and applied to 4 cases of Legg-Perthes' disease as one of conservative managements. The brace was modified into “abduction-internal rotation functional long leg brace” in cases of Legg-Perthes disease. In 1 cases of post-infectious subluxation of the hip, the stability was maintained by use of the brace. With this experience, it is expected that the ischial weight-bearing functional long leg brace may be applied to other pathologic conditions of the lower extremity in which weight-bearing is to be avoided or guarded.
Braces ; Gait ; Hip ; Humans ; Hygiene ; Immobilization ; Joints ; Knee Joint ; Leg ; Legg-Calve-Perthes Disease ; Lower Extremity ; Orthopedics ; Seoul ; Skin ; Thigh ; Tibia ; Weight-Bearing

Osteochondritis dissecans is a joint affection characterized by partial or complete detachment and necrosis of cartilage fragment and underlying bone from the articular surface. It is seen frequently in knee, elbow and ankle joints. Young males are most commonly affected. Also it develops not infrequently in children, but never in a child under 4 years of age. A case of osteochondritis dissecans which occurred in a 6-year-old boy is reported here. The patient had been sufferred from pain, swelling and progressive limitation of motion of the left knee. X-ray showed shadows like loose bodies on posteromedial aspect of the left knee. Clinically, possibility of tuberculous arthritis could not be rulled out. Loose bodies were removed, and almost detached ostechondral fragment was excised. At post-operative second week, crutch walking and physiotherapy were recommended.
Ankle Joint ; Arthritis ; Cartilage ; Child ; Elbow ; Humans ; Joints ; Knee ; Male ; Necrosis ; Osteochondritis Dissecans ; Osteochondritis ; Walking

No abstract available in English.
Scoliosis

Infectious mononucleosis is a common systemic illness primarily invloving children and young adults and due apparently to the Epstein-Barr virus (EBV). Although the typical case is easily recogniged, unusual manifestations or serious complications occur frequently enough to pose problems in diagnosis and management. Neurologic complications are rare(1%). These nerologic complications may actually precede the full clinical picture or present as the sole manifestation of the illness, thereby causing a confusing clinical picture and delay in diagnosis. We have experienced a case of infectious mononucleosis associated with facial nerve palsy in a 22-months old female. She was admitted to the hospital with the fever, right facial nerve palsy and hepatosplenomegaly. The diagnosis was made on the clinical findings, immunologic findings and the typical findings of peripheral blood smear. On peripheral blood smear, atypical lympocytes with relative lympocytosis were seen. On Immunologic study EBV anti-VCA IgM titer was 1:20 and the IgG titer was 1:40. To our knowledge, this is the first report in Korea. So, we report this case and the brief literatures of infectious mononucleosis associated with neurologic complications and other viral diseases which caused facial nerve palsy.
Child ; Diagnosis ; Facial Nerve* ; Female ; Fever ; Herpesvirus 4, Human ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Infant ; Infectious Mononucleosis* ; Korea ; Paralysis* ; Virus Diseases ; Young Adult

Acute tubular necrosis has been rarely reported as a complication of typhoid fever in the literature. We experienced four cases of acute tubular necrosis associated with typhoid fever in children. Patients showed significant titer of widal reaction associated with acute renal failure and one of them rised in 2 months after onset of clinical symptoms. Renal biopsy findings were compatible with acute tubular necrosis and immune complexes were not deposited in the glomeruli. Clinical and urinary findings were normalized by hemodialysis and antibiotic therapies. In conclusion, close longterm follow up of widal titer is mandatory in acute tubular necrosis, especially when associated with high fever.
Acute Kidney Injury ; Antigen-Antibody Complex ; Biopsy ; Child ; Fever ; Follow-Up Studies ; Humans ; Necrosis* ; Renal Dialysis ; Typhoid Fever*