Angioedema is a disorder characterized by well-demarcated nonpitting edema involving the tongue, floor of the mouth, larynx, lips, and face. The incidence of angiotensin converting enzyme(ACE) inhibitor related angioedema has been reported to be about 0.1% to 0.2%, and the time of onset is usually during the first week of therapy. These ACE inhibitors include captopril, enalapril, and lisinopril. A 53-year old man with an 8 month history of hypertension previously controlled with atenolol, was presented to the dermatologic department with angioedema of the face and tongue. He had begun therapy with captopril one day before this episode. Even though he was treated with epinephrine and methylprednisolone sodium succinate, the edema gradually progressed and finally dyspnea developed. He was urgently intubated and treated with steroids and pheniramine maleate in the intensive care unit. The edema resolved after 24 hours.
Angioedema* ; Angiotensin-Converting Enzyme Inhibitors ; Angiotensins ; Atenolol ; Captopril* ; Dyspnea ; Edema ; Enalapril ; Epinephrine ; Humans ; Hypertension ; Incidence ; Intensive Care Units ; Larynx ; Lip ; Lisinopril ; Methylprednisolone Hemisuccinate ; Middle Aged ; Mouth ; Pheniramine ; Steroids ; Tongue

No abstract available.
Spinal Muscular Atrophies of Childhood*

The present study is aimed to investigate the clinical and pathologic features of GI lymphomas and the immunocytochemical characteristics, using 71 cases of primary GI lymphomas examined at the Department of Pathology, Yonsei University College of Medicine, Youngdong Serverance Hospital and Yonsei University Wonju College of Medicine from January, 1976 to December, 1985. Results obtained were as follows: 1) Sixty eight cases were Non-Hodgkin's lymphoma and 3 Hodgkin's lymphoma. Surgical resection was done in 58 patients and surgical biopsy in 13. 2) The primary sites of the tumors were stomach, small intestine, ileocecal area and large intestine in a descending order of frequency. 3) The mean age at the time of diagnosis was 45 years. The tumor of the small intestine was generally detected in the younger age and that of the large intestine in the older age. The overall male to female ratio was 1.9 : 1. 4) Abdominal pain was the most common presenting symptom irrespective of the sites of the tumor. Duration of the symptoms were evenly distributed from hours to 12 monthes. 5) The size of the tumors was less than 10 cm in the largest dimension in most of the cases (79.3%). The gross types of the GI lymphomas were ulcerative (27.6%), polypoid (20.7%), multinodulated (17.2%) and diffuse thickening. The rate of lymph node involvement was 44.4% in tumors confined to the serosa, while 82.6% in tumors with serosal penetrations. 6) Classification of 68 cases of Non-Hodgkin's lymphoma according to Working Formulation revealed 50 cases of intermediate grade, 13 cases of high grade and 5 cases of low grade. According to Rappaport classification, all were diffuse types, among which diffuse histiocytic type was the most common. According to Lukes-Collins classification, 60 cases were B cell types, 4 cases genuine histiocytes types and 3 cases T cell types. Most of the gastric and intestinal lymphomas belonged to the follicular center cell (small and large cleaved, large cleaved) types. 7) Immunoperoxidase stains were done in 55 cases of malignant lymphomas. Thirty (65.9%) of 47 B cell lymphomas revealed a positivity for B cell markers. Twenty three (48.9%) of them showed a monoclonality, in which kappa/IgG pattern was most frequently demonstrated. Four cases of true histiocytic lymphomas were positive for alpha-1-antichymotrypsin and lysozyme, the former of which was seemed to be a more sensitive marker for histiocytic differentiation. An attempt was made to evaluate the significance of the degree of reactive histiocytic infiltrates within the lymphoma in relation to the progosis, but correlation could not be made because of the limited cases.
Female ; Male ; Humans ; Biopsy

Juvenile xanthogranuloma(JXG) is a a benign histiocytic proliferative disorder most frequently seen in infants. The usual course is self-limited, but it may be a manifestation of juvenile chronic myeloid leukemia(JCML). In addition to JXG, some patient with JCML may have multiple cafe-au-lait spots and a family history of neurofibromatosis(NF). The association of JXG and cafe-au-lait spots in patients with JCML is of interest in light of description of otherwise healthy children with JXG who also had cafe-au-lait spots. We report herein a case of JXG in 18-month-old boy who also had multiple cafe-au-alit spots, which predated the JCML.
Cafe-au-Lait Spots ; Child ; Humans ; Infant ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Male ; Neurofibromatoses* ; Xanthogranuloma, Juvenile*

No abstract available.
Female* ; Humans ; Prune Belly Syndrome*

Thirty cases of rhabdomyosarcoma in patients under the age of 15 years were reviewed retrospectively to find out characteristics of histologic subtypes related to prognosis. Histologic diagnosis were embryonal in 21(70%), alveolar in 7(23%) and mixed type in 2(7%). Cellular anaplasia was noted in 6 cases. Round to polygonal cells with abundant eosinophilic cytoplasmic rim or globules were most characteristic cytologic feature or rhabdomyosarcomas, being followed by myofibrils. Myxoid and edematous stroma in embryonal type and alveolar pattern in alveolar type wee nost characteristic growth pattern. Immunohistochemically, tumors were positive for desmin, actin and myoglobin in 97%, 27%, and 40%, respectively. Consequently, characteristic cytologic features are important to differentiate rhabdomyosarcomas form other kinds of small round cell tumor and growth pattern is more critical in categorizing histologic subtypes. Desmin is far more useful than actin or myoglobin in the diagnosis of rhabdomyosarcoma.
Child ; Male ; Female ; Humans

BACKGROUND: Renal dysplasia is the abnormal development of the kidney. The condition is usually detected in childhood along with other urinary tract anomalies, but can remain unnoticed until adulthood. It was recently reported that a PAX2 gene mutation plays a major role in the development of renal dysplasia. The aim of this study was to examine the expression of PAX2 in dysplastic kidneys of children and adults. METHODS: A total of 30 cases diagnosed with renal dysplasia after a nephrectomy were examined. PAX2 expression was evaluated using immunohistochemistry. Apoptosis was detected using an Apop Tag detection kit. RESULTS: In the dysplastic kidneys, there was strong PAX2 expression in the epithelia of the primitive ducts in both children and adults, but the degree was significantly lower in adults (p=0.007). However, the mesenchyme surrounding the primitive ducts of children showed stronger staining for the smooth muscle actin antibody and trichrome than the adults. The apoptosis index was significantly higher in the primitive duct epithelia than in the surrounding normal collecting duct epithelia (p=0.000). CONCLUSIONS: PAX2 is overexpressed in the primitive ducts of renal dysplasia, which is sustained until adulthood and is associated with increased apoptosis. However, a decrease in PAX2 expression in the dysplastic epithelia and mesenchymal cuff of adults suggest a gradual regression of the dysplastic elements with time.
Actins ; Adult ; Apoptosis ; Child ; Humans ; Immunohistochemistry ; Kidney ; Mesoderm ; Muscle, Smooth ; Nephrectomy ; Urinary Tract

No abstract available.
Autoantibodies* ; Humans ; Nervous System* ; Trauma, Nervous System*

SUMMARY OF LITERATURE REVIEW: Many authors haute described the association between lumbar disc degeneration and segmental instability but it has not been delineated in detail. OBJECTIVES: To compare the MRI assessment of disc degeneration with the conventional plain X-ray evaluation of the intervertebral disc, in order to study lumbar segmental instability. MATERIALS AND METHODS: In 75 patients with low back pain and/or sciatica, we analyzed disc space height, angular displacement, and horizontal displacement on plain radiographs of the lumbar spine. These parameters were compared with the grade of disc degeneration as evaluated by magnetic resonance Imaging. STUDY DESIGN: We analyzed the association between grade of disc degeneration as evaluated by magnetic resonance imaging assessment and segmental instability as evaluated by plain radiographs of the lumbar spine. RESULTS: Disc space height was decreased In proportion to the grade of disc degeneration. Angular displacement was increased according to the grade of disc degeneration, but significantly less with severe degeneration, accompanied by a tendency to stabilization of the motion segment. Horizontal displacement was not correlated with the grade of disc degeneration CONCLUSIONS: The incidence of lumbar segmental instability increased in proportion to the grade of disc degeneration but significantly decreased with severe disc degeneration.
Humans ; Incidence ; Intervertebral Disc ; Intervertebral Disc Degeneration* ; Low Back Pain ; Magnetic Resonance Imaging ; Sciatica ; Spine

No abstract available.
Hepatolenticular Degeneration*