Hepatoblastoma is a rare malignant liver tumor found in children. Its biological characteristics and prognostic factors have not been well known. We investigated 29 cases of hepatoblastoma, registered in university hospitals in Seoul from 1984 to 1996. By the immunohistochemical method, p53, Waf-1 (p21), bcl-2, heat shock protein 70 (hsp70), c-jun, transforming growth factor-alpha (TGF-alpha) expressions were studied. Those data were compared with clinico-pathologic features; age, sex, tumor size, tumor stage and histologic subtypes. Expression of p53 and bcl-2 were each observed separately in single cases. Expression of c-jun was more frequently noted in patients at higher stages. Expression of TGF-alpha decreased in the order of pure fetal, mixed, embryonal and small cell anaplastic subtypes. Cumulative survival rate was lower in females than in males and in patients with a higher tumor stage. According to histologic subtypes, survival rates decreased in the order of pure fetal, mixed, embryonal and small cell anaplastic subtypes. Survival rate was lower in patients with c-jun expression. Group of TGF-alpha labelling index under 19 showed a lower survival rate than that over 19. In conclusion, we found that tumor associated proteins, c-jun and TGF-alpha, are closely related to the prognosis of hepatoblastoma but p53 and bcl-2 may not be related to it.
Child ; Female ; Hepatoblastoma* ; Hospitals, University ; HSP70 Heat-Shock Proteins ; Humans ; Liver ; Male ; Population Characteristics ; Prognosis ; Seoul ; Survival Rate ; Transforming Growth Factor alpha

Dystrophic epidermolysis bullosa is a rare, chronic non-inflammatory bullous disease, which easily forms bullae by minor mechanical trauma or spontaneously, is inherited either in an autosomal dominant or autosomal recessive fashion. We report herein two cases which presented with bullae, erosions and ulcers on extremities, buttock, chest, abdomen and face and loss of all nail since birth in two sisters. Bulla occured bencath the basal lamina histopathologically, anchoring fibrils were almost absent on electron miaoscopy in both cases. The two sisters represented dystrophic epidermolysis bullosa considering the absence of family history inheritcd in an autosomal dominant fashion and the clinical, histological and electronmicroscopic findings.
Abdomen ; Basement Membrane ; Buttocks ; Epidermolysis Bullosa Dystrophica* ; Extremities ; Humans ; Parturition ; Siblings* ; Thorax ; Ulcer

No abstract available.

No abstract available.

No abstract available.
Erythroblastosis, Fetal* ; Infant, Newborn

Tetanus is a rare disease in the developed countries, including Korea, as a result of nearly universal active immunization. Because many physicians have little experience with its diagnosis and management, misdiagnosis and therapeutic delay may result in catastrophic consequences. We report one case of generalized tetanus. A previously healthy 35-year-old man was admitted to the emergency department because of stiffness on jaw and neck. board-like abdomen, extended and rigid both legs, chest tightness, and dyspnea. Seven days before admission, he was injured on the right thing at work. In the emergency department, he was given 5,000 units of human tetanus immunoglobulin intramuscularly, as well as diazepam 2 mg/hr intravenously. On the second hospital day, tracheostomy was performed. Ten days later, his condition was improved. Twenty eight days after admission, he was discharged without any complication.
Abdomen ; Adult ; Developed Countries ; Diagnosis ; Diagnostic Errors ; Diazepam ; Dyspnea ; Emergency Service, Hospital ; Humans ; Immunoglobulins ; Jaw ; Korea ; Leg ; Neck ; Rare Diseases ; Tetanus* ; Thorax ; Tracheostomy ; Vaccination

No abstract available.

Cutaneous metastasis from renal cell carcinoma is not unusual, occurring in 3 to 7%, although it may be overlooked. We report a case of metastatic renal cell carcinoma in a 74-year-old man who was presented with a 1.5 x 1.5 x 0.5 cm-sized nodule on the right zygoma for 3 months. A skin biopsy demonstrated the typical clear cell adenocarcinoma with PAS(+) granules in the cytoplasm and honeycombed or glandular configuration, compatible with metastatic renal cell carcinoma. Kidney ultrasonogram and abdomina1 CT scanning showed a large cystic mass on the upper pole of the left kidney. Furthermore radiologic studies revealed multiple metastatic lesions in the lung, liver and brain. He died 4 months later.
Adenocarcinoma, Clear Cell ; Aged ; Biopsy ; Brain ; Carcinoma, Renal Cell* ; Cytoplasm ; Humans ; Kidney ; Liver ; Lung ; Neoplasm Metastasis ; Skin ; Tomography, X-Ray Computed ; Ultrasonography ; Zygoma*

It is well-known that the infection of bone and joints could produce the shortening by destroying the articuiar cartilage and growth plate, or the lengthening hy increased hlood circulation to the epiphyseal and metaphyseal regions of long bone. Since 1985, we have treated total l7 cases of children s pyogenic or tuberculous children s knee with early arthrotomy and continuous irrigation etc. and followed their long hone growth for average 53 months after surgical intervention. Five patients (group I ) were early treated within 2 weeks and twelve patients(group 3 ) were delayed to treat their knees for more than 2 weeks of symptom development. Total 11 patients out of 17 cases(64.7%) (2 cases of group 1 pyogenic arthritis, and 1 case of group l and 8 cases of group II tuberculous arthritis) revealed growth acceleration. Femoral lengthening was much more greater than that of tibial growth, and the size of patella, distal femoral and proximal tibial epiphysis which were measured by computerized digitizer were also significantly larger than that of opposite knee. The total limb length was increased hy average 7mm in 6 months, l4mm in 1 year and 3mm in 2 years after operation. These results suggest the pyogenic and tuberculous arthritis in children s knee should he treated as early as possible after symptoms developed, and followed them to trace their limh inequality for at least 2 years after surgery. That means the early arthrotomy for tuberculous knee or even in pyogenic arthritis can preserve normal articular cartilage and can also prevent the early epiphyseal closure hy infection. and can get the limh lengthening by increased blood circulation around the knee joint.
Acceleration ; Arthritis ; Blood Circulation ; Cartilage ; Cartilage, Articular ; Child* ; Epiphyses ; Extremities ; Growth Plate ; Humans ; Joints ; Knee Joint* ; Knee* ; Lower Extremity* ; Patella ; Socioeconomic Factors

No abstract available.
Heel* ; Molluscum Contagiosum* ; Weight-Bearing* ; Zea mays