Neurofibromat osis is a familial discase with widespread manifestations such as lesions of the skin, tumors of the central and peripheral nervous system and abnormalities of bone. The presence of at least two of the following features was considered for diagnostic criteria; positive family history, positive blopsy, a minimum of six cafe-au-lait spots each with a diameter of at least 1.5 cm and multiple subcutaneous nodules. From January 1971 to December 1979, 17 patients of neurofibromatosis and 30 patients of neuro- fibroma treated at Yonsei Medical Center were clinically analized for the study and the results obtained from this study were as follows; 1. Positive family history was obtained In 2 per cent of cases with neurofibromatosis. 2. Cafe-au-lait spots or tumors were the most common findings in neurofibromatosis. The bone changes were presented in 8 patients(45 per cent) and scoliosis has been In 62.5 per cent of them. 3. The location of the neurofibroma was variable. Central nervous sustem was involved in 40.4 per cent and peripheral nervous system in 59.4 per cent. 4. Myelography and determinations of the cerebro-spinal fluid protein concentration led us to a correct diagnosis of the neurofibroma which involved the spinal cord. For the treatment of the neurofibroma, 9 out of 30 patients were performed laminectomies and excision of the intradural masses, and one of them was flxed the spinous processes with a plate and wires after laminectomy for prevent spinal instability. 5. in 3 patients with progressive scoliosis, the best results were obtained with early Harrington instrumentation and posterior fusion.
Cafe-au-Lait Spots ; Chymopapain ; Clinical Study ; Diagnosis ; Fibroma ; Humans ; Laminectomy ; Myelography ; Neurofibroma ; Neurofibromatoses ; Peripheral Nervous System ; Scoliosis ; Skin ; Spinal Cord

Dermstomyositis is a disease mainly of the skin, muscle and blood vessels in which erythematous and edematous changes in the skin are associated with muscle weakneas and inflammation. In adult the disease is commonly associated with underlying malignancy or reticulosis. A 23 year-old male had generalized weakness, intermittent fever, myalgia and purplish red patches on the face. Diagnosis of dermatomyositis was established by clinical manifestations, electromyogram, histologic findings of skin and muscle. He was treated with systemic steroid, with some improvement. Seven months after the onset of dermatomyositis, ecchymoses was noted on the eyelids and forearms. Bone marrow aspiration showed many immature lymphoblasts, characteristic of acute lymphocytic leukemia, He was treated with systemic steroid and vincristine, but no favorable effect was noted. He died of sepsis due to retal abscess on the 34th day in the hospital
Adult ; Male ; Female ; Humans

A case of a clinical syndrome of selective hereditary complete IgA deficiency with partial T-lymphocyte inactivation is reported. A 14 year old boy was admitted with chjef complaint of persistent wide shallow ulcerated non-vesicular patches on the skin folds of whole body, chronic upper respiatory infections and gastointestinal upsets. General appearance showed us as if the undemourished or malabsorption status had been accompanied. The duration of illness was about 10 years under the incorrect or mistaken diagnosis and skin lesions have incompletely responded to the treatment. Peripheral T-lymphocyte determination was carried out by the method of Wybran to be tumed out active T-cell 14%, total T-cell 48%. Tuberculin test showed negative with 5 TU. Immunoglobulin values were respectively as follows: IgG 325 mg%, IgM 60 mg%, and IgA trace. And we diagnosed this case as selective IgA defiency with partial T-lymphocyte inactivation. We started the immunochemotherapy with tuberculo-protein complex, Tubercin-3 and antibictics for 2 months. After 2 months with above regimen. the skin lesions were markedly improved. T-cell count also retumed to the normal limit(active 35%, total 59%) and levels of TgG and IgM raised(IgG 617.5 mg%, IgM 75mg%), but IgA still showed trace amount, Tuberculin test tumed out positive.
Adolescent ; Dermatitis* ; Diagnosis ; Humans ; IgA Deficiency* ; Immunoglobulin A* ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins ; Lymphocytes* ; Male ; Skin ; T-Lymphocytes ; Tuberculin Test ; Ulcer

Transient synovitis is characterized by the development of pain arising from the hip and often felt in the thigh or knee in children between the ages of 2 and 12 years. The benign self-limiting nature of this condition has made it difficult to establish the caus'e. However, the condition is generally conceded to be the commonest cause of a painful hip in childhood. The disease is of interest, not because of its disabling condition, but because of the difficulty in differentiating it from more serious diseases of the hip, such as tuberculosis, osteomyelistis, or Legg-Perthes disease. The author had studied the clinical symptoms and signs, roentgenologic findings, and treatment of the 34 patients who had admitted to Severance Hospital Yonsei Medical Center in Seoul, Korea from January 1970 to December 1979, under the diagnosis of the transient synovitis of the hip and summerized the results as follows; 1. There was probably associated with predisposing factors such as infection and allergy. 2. Among the 34 patients, 28 cases(82.4%) were male and 6 cases(17.6%) were female. The most prevalent age were between 6 and 10 years old. 3. Pain and limitation of motion of the hip joint were the most common symptoms. In 56.5% of the patients, roentgenographic findings were positive. In 50% of the patients, the erythrocyte sedimentation rates were increased. 4. The treatment was conservative including bed rest, skin traction on the affected leg, non-weight bearing, antibiotics, and sedatives. Most of all patients had improved symtoms and signs within two weeks of treatment. 5. The course of this condition was short and benign with complete resolution. The occasional hip with chronic or recurrent symptoms could be distinguished from Legg-Perthes disease by the short history, normal radiographs, and the complete resolution.
Anti-Bacterial Agents ; Bed Rest ; Blood Sedimentation ; Causality ; Child ; Clinical Study ; Diagnosis ; Female ; Hip Joint ; Hip ; Humans ; Hypersensitivity ; Hypnotics and Sedatives ; Knee ; Korea ; Leg ; Legg-Calve-Perthes Disease ; Male ; Seoul ; Skin ; Synovitis ; Thigh ; Traction ; Tuberculosis

The antihypertensive effect of captopril was evaluated in 15 patients with mild to moderate essestial hypertension(Mean age : 55, Mean blood pressure : 171/102mmHg). Captopril was administered 25 to 50mg twice daily with or without hydrochlorothiazide by mouth according to our protocol which was presented in the text. All the patients were followed up to 12 weeks. Captopril with or without hydrochlorothiazide significantly lowered the systolic and diastolic pressure in almost all patients. The mean systolic and diastolic pressure were reduced to 134/86mmHg(-37/-16mmHg ; -22/-16%)(p<0.001). Heart rate did not change significantly. No unwanted effects were observed. We conclude captopril has exellent antihypertensive effect in most patients with mild to moderate essential hypertension.
Blood Pressure ; Captopril* ; Heart Rate ; Humans ; Hydrochlorothiazide ; Hypertension* ; Mouth

Expression of the nm23 and E-cadherin proteins has been studied in a number of tumors. Reduced expression of the nm23 and E-cadherin proteins seems to be associated with metastasis or disease progression in some tumors, including breast carcinoma. To assess the role of nm23 and E-cadherin in tumor differentiation and metastasis of breast carcinoma, immunohistochemical staining for the nm23 and E-cadherin proteins was performed in paraffin embedded tumor samples from 86 breast carcinomas. The results were as follows: 1) Expression of the nm23 protein in breast carcinoma was strong positive in 32 cases (37.2%), weak positive in 26 cases (30.2%), and negative in 28 cases (32.6%) of the cases. Expression of the nm23 protein in breast carcinoma decreased according to histological grade and lymph node metastasis, but was not statistically significant. 2) Expression of the E-cadherin protein in breast carcinoma was strong positive in 50 cases (58.1%), weak positive in 24 cases (27.9%), and negative in 12 cases (14%) of the cases. Expression of the E-cadherin protein in breast carcinoma decreased according to histological grade and lymph node metastasis, but was not statistically significant. 3) There was a statistically significant correlation between the expression of the nm23 protein and the E-cadherin protein in breast carcinoma (p<0.05). These results suggest that the expression of the nm23 and E-cadherin proteins is related to tumor differentiation, and may also be an useful prognostic factor in breast carcinoma.
Breast Neoplasms* ; Breast* ; Cadherins* ; Disease Progression ; Lymph Nodes ; Neoplasm Metastasis ; Paraffin

We examined 984 objects for exercise stress test, using CASE I(computer assisted system for exercise). They exercised according to Bruce's protocol, Among them, the mal were 613 and the female were 371. (Male:Female;17:1) The 5th and 6th decades occupied 61% of total objects. The purpose of test was evaluation of chest pain in 84% of total cases. The most frequent end point of test was dyspnea(42%) and chest pain was observed in 13% of total cases. Most of all objects above 8-9 METS. Positive results of exercise stress tert were obtained in 23%. As complications, ventricular tachycardia occurred in 7 cases, supraventricular tachycardia; 8, high degree VPB's; 6, sudden cardiovascular collapse;15, The complication rate was 0.36% but morbidity and mortality were 0. All complicated cases recoved spontaneously only by rest.
Chest Pain ; Electrocardiography* ; Exercise Test ; Female ; Humans ; Mortality ; Tachycardia, Supraventricular ; Tachycardia, Ventricular

No abstract available.
Cross Infection*

Primary extranodal lymphoma of the liver is very rare, approximately thirty-one cases having been reported in the literature. We report one case of primary malignant lymphoma of the liver in a 26-year-old female, who was presented with palpable abdominal mass on the epigastrium for about 40 days. Laboratory findings revealed no specific abnormalities. Peripheral lymph nodes or spleen were not palpable. An abdominal ultrasonogram revealed a huge mass involving the entire left lobe of the liver. Left lateral segmentectomy of the liver was done. The resected lateral portion of left lobe of the liver showed a large solitary mass, 12 cm in the greatest dimension, with a yellowish gray fleshy solid cut surface. Though light microscopic feature was compatible with primary malignant lymphoma, diffuse large cell type, intermediate grade by the working formulation, undifferentiated carcinomas including hepatocellular carcinoma, plasmacytoma, and pleomorphic sarcomas could not be completely ruled out. Thus, marker studies and electron microscopic examination were performed. Immunoperoxidase stains for common leukocyte antigen was positive, and the Leder stain for myeloid granule was negative. Electron microscopic stuids revealed findings of neoplastic lymphoid cells, consistent with malignant lymphoma. Primary lymphoma of the liver has only rarely been reported, and its natural history is unclear. Many of the reported cases have been large cell lymphomas, as in this case.
Female ; Humans ; Carcinoma, Hepatocellular

No abstract available.
Blood Donors* ; Humans ; Seroepidemiologic Studies*