No abstract available.
GRB2 Adaptor Protein*

In pili torti, the affected Eair shaft is flattened and twisted through 180 degrees on its own axis. The involved hairs are dry, thin, brittle, and break off easily. Congenital pili torti may occur as an isolated phenomenon or may occur in association with other abnormalities. Acquired pili torti is usually associated with some sort of scarring process in the scalp itself. We present a case of congnital pili torti without any other abnormalities in a 14 year-old female. The pedigree of her fariiily was consistent with the inheritance of congenital pili torti as an autosomal dominant trait.
Adolescent ; Axis, Cervical Vertebra ; Cicatrix ; Female ; Hair ; Humans ; Pedigree ; Scalp ; Wills

Refractory deformity in ankylosing spondylitis is caused by loss of normal lordotic curvature in lumbar spine. For the correction of deformity, monosegmental osteotomy, intracorporal decancellation and multisegmental osteotomy are used. Among them, multisegmental osteotomy is reported to be safe because of its small correction amount at each level. Since 1989, authors treated 5 cases of ankylosing spondylitis with severe kyphotic deformity by multisegmental osteotomy and transpedicular instrumentation. All were young males between 22 and 35 years of age. Preoperative kyphotic deformity was 80°, 105°, 72°, 35°, 55° (Av. 70°) and amount of correction was 55°, 105°, 72°, 20°, 40° (Av. 58°) respectively. Levels for osteotony were 4 to 8 segments and correction at a level was 5° to 13° (Av. 8.3°). Normal standing upright posture and vision for straight forward were obtained in all. Instrumentation was Zielke in three Cotrel-Dubousset in two. One case of Zielke instrumentation showed rod failure. However, all showed solid bony union without any loss of correction or pseudoarthrosis. From the above experience, multisegmental osteotomy for the treatment of kyphotic deformity in ankylosing spondylitis was believed to be a safe and effective method of treatment.
Congenital Abnormalities ; Humans ; Male ; Methods ; Osteotomy ; Posture ; Pseudarthrosis ; Spine ; Spondylitis, Ankylosing

The electrocardiographic and echocardiographic analysis was made on 23 patients with atrial septal defect of ostium secundum type, who were confirmed by right heart catheterization under admission at hanyang University Hospital from january 1979 to July 1981. Following results were obtained: 1) In the 23 patients, 13 were male and 10 female. Their ages ranged from 6 to 45 years, mean age being 19.9 years. 2) In 18 patients(78.3%), the QRS axis was between +90degrees and +150degrees. No significant correlations could be demonstrated between QRS axis and some hemodynamic parameters(right ventricular systolic pressure, pulmonary artery mean pressure and pulmonic to systemic flow ratio), and also, between QRS axis and the size of right ventricular dimension index. 3) Right ventricular outflow tract hypertrophy, right ventricular hypertrophy and complete right bundle branch block pattern in Lead V3R or V1were observed in 95.7% of ostium secundum defect. No significant correlations could be found between types of QRS complexes and some hemodynamic parameters(right ventricular systolic pressure, pulmonary artery mean pressure and pulmonic to systemic flow ratio), and also, between types of QRS complex and the size of right ventricular dimension index. 4) The mean amplitude of S wave in precordial Lead V6was 7.6mm. There was a definite correlation between the amplitude of S wave in Lead V6and right ventricular systolic pressure. No significant correlations could be demonstrated betwen the amplitude of s wave in Lead V6and some hemodynamic parameters(pulmonary artery mean pressure and pulmonic to systemic flow ratio), and also, the size of right ventricular dimension index. 5) Right ventricular dimension index was increased in all cases but one. A significant correlation was found between the size of right ventricular dimension index and pulmonary artery mean pressure, whereas no significant correlation could be demonstrated between the size of right ventricular dimension index and pulmonic to systemic flow ratio. 6) 95.7% of 23 patients with ostium secudum defect showed paradoxical septal motion was proved not to be associated with other congenital heart disease by cardiac catheterization. The pulmonic to systemic flow ratio in groups of septal motion type A was significantly increased than that of normal septal motion group, whereas no significantl relation between types of septal motion and the size of right ventricular dimension index was observed.
Arteries ; Axis, Cervical Vertebra ; Blood Pressure ; Bundle-Branch Block ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography* ; Electrocardiography* ; Female ; Heart Defects, Congenital ; Heart Septal Defects, Atrial* ; Hemodynamics ; Humans ; Hypertrophy ; Hypertrophy, Right Ventricular ; Male ; Pulmonary Artery

Bowens disease is generally regarded as a premalignant dermatosis. If untreated, 3% to 5% of patients may develop squamous cell carcinoma. However, sebaciou carcinoma arising from Bowens disease is very rare. We presented a case of quarnous cell carcinoma and sebaceous circ s disease in a 68 year-old male. He had multiple bowenoid skin lesi nsties. A bean-sized nodule as developed on the bowenoid lesion of the he had a large yellow crust.ed exudative tumor on the Rt. thigh. We took a biopsy specimen of these three discrete lesions. The nu lipid stain of frozen section revealed Bowens disease, squarnous concllnoma arising from Bowen on the trunk and extremi Rt. lower abdomen. Almost of routine histology and carcinoma, and sebaceous carcinoma, respectively.
Abdomen ; Aged ; Biopsy ; Bowen's Disease ; Carcinoma, Squamous Cell* ; Frozen Sections ; Humans ; Male ; Skin ; Skin Diseases ; Thigh

A tender, non-healing ulcer of 3 months' duration developed on the tongue tip of a 51-year-old male with active pulmonary tuberculosis. Acid-fast bacilli were grown in the tissue and sputum cultures. The patient was anergic to PPD skin test before the treatment and IL-2 productivity was significantly decreased. Sections from the tongue tip showed ulceration, infiltration of neutrophils and tuberculoid granulornas accompanying caseation necroses in the dermis. The ulcer healed after administration of anti-tuberculosis medication for 2 months.
Dermis ; Efficiency ; Humans ; Interleukin-2 ; Male ; Middle Aged ; Necrosis ; Neutrophils ; Skin Tests ; Sputum ; Tongue* ; Tuberculosis* ; Tuberculosis, Pulmonary ; Ulcer

Normal C3WHeN strain mice exposed to topical 8inethoxypsomlen plus long wave ultraviolet (PUVA) showed a reduction in contact hypersensitivity, (CH) which was localized to the skin in the area of PUVA treatment (local suppression), whereas systemic PUVA treatment caused diffuse suppression of CH reaction, regardless of the application site of 2,4-dinitro-1-fluorobenzene (DNFB). There seem to be two different mechanisms responsible for CH reduction by PUVA. Local suppression by topical PUVA treatment was thought to be a result of blocking the afferent phase of immune response, it was associated with a lack of CH effector cells in the peripheral lymph nodes and could not be reversed by indomethacin treatment. Diffuse suppression induced by systemic PUVA treatment seemed to be associated with blocking of egress of effector cells from the regional lymph nodes, this depressed CH response was prevented when indomethacin was administered before PUVA treatment.
Animals ; Dermatitis, Contact* ; Indomethacin ; Lymph Nodes ; Mice ; Skin

No abstract available.
Amputation* ; Fingers*

The immunocompetence is important not only to kill the neoplastic cells but also to keep the neoplastic cells from growing further. T lymphocyte is plays the most important role in maintaining the tumor immunity efficiently. T lymphocyte has its specific functions depending in the subset of T lymphocytes. The author analyzed the T lymphocyte subsets in 31 brain tumor patients using anti-CD3, anti-CD4, anti-CD8 monoclonal antibodies and flow cytometry to determine the immunological status of brain tumor patients. All CD3, CD4 and CD8 subsets were reduced in both benign and malignant brain tumor patients but more signigicantly reduced in malignant tumor group. But in benign tumor group, the subtypes of T lymphocytes were not so different from those of normal healthy controls except the pituitary tumor patients, who showed the significant decrease in all the subtypes. In malignant tumor group, each subtype was signigicantly reduced and CD8 subtypes was markedly reduced in metastatic tumor patients, These analyses were considered to have the possibility to be contributable to planning the further immunotherapy and also the possibility to moniter the brain tumor patients clinically.
Antibodies, Monoclonal ; Brain Neoplasms* ; Brain* ; Flow Cytometry ; Humans ; Immunocompetence ; Immunotherapy ; Lymphocytes ; Pituitary Neoplasms ; T-Lymphocyte Subsets* ; T-Lymphocytes*

The high incidence antigert Jr" was first identified in 1970 by Stroup and Macllroy. Anti-Jr~ was immune antibodies developed by transfusion or pregnancy and occasionally cause hemolytic disease of the newborn and transfusion reaction, but these usually mild. We are reporting the first case of anti-Jra in Korea, which was identified in pregnant woman with transfusion history. The 35 year old pregnant woman(G6P4L1D3A1) admitted to treat for incompetent internal os of cervix on department of Obstetrics and Gynecology. Prenatal irregular antibody screening was negative. One unit of RBC was transfused at 21 gestational weeks for correction of anemia. One week later, irregular antibody was detected in her serum. The antibody reacted best by indirect antiglobulin test and panagglutinated all identified cells. The titer was 1 : 8. The antibody was identified as anti-Jr by Dr. Osaka Red Cross Center. Her phenotype of Jra was Jr(a-), but there was no Jr(a-) person in her family. She might have anti-Jra in her serum with undetectable level due to multiple pregnancies. In this case, the development of anti-Jr was stimulated by one unit of RBC transfusion with anamnestic reaction. The titer of anti-Jr was gradually reduced during pregnant period. She delivered at 37 gestational weeks by cesarian section. The baby was clinically well at birth and typed as Jr(a+).
Adult ; Anemia ; Antibodies ; Blood Group Incompatibility ; Cervix Uteri ; Coombs Test ; Female ; Gynecology ; Humans ; Incidence ; Infant, Newborn ; Korea ; Mass Screening ; Obstetrics ; Parturition ; Phenotype ; Pregnancy ; Pregnancy, Multiple ; Pregnant Women* ; Red Cross