No abstract available.
Marfan Syndrome*

PURPOSE: The proximal femoral morphology of normal Koreans was studied by radiographs. MATERIALS AND METHODS: Proximal femoral morphology and ratio of lever arm in abductor muscle in 101 normal adults were measured and their correlations were comparatively analyzed. RESULTS: The femoral head diameter was 48.7+/-4.0 mm. The femoral neck shaft angle was 127.8+/-5.2 degree. The medial trabeculae angle was 20.0+/-1.2 degree. The femoral offset was 36.5+/-3.9 mm. Also, the tip of the greater trochanter relative to the level of femoral head center lay higher in 91 of 101 subjects (90.1%), upon examination and in average, located 3.8+/-3.4 mm higher. The ratio of the abductor lever arm to the distance between the femoral head center and the body midline was 1: 2.1+/-0.2. CONCLUSIONS: The relationship between the tip of the greater trochanter of femur and the center of femoral head appeared to have wide individual differences with 0.89 coefficient of variation. Therefore, the tip of the greater trochanter was an unreliable anatomical indicator of femoral head center. The femoral neck shaft angle and the length of the medial trabeculae showed a significant correlation (P=0.001). However, a significant negative correlation existed between the femoral neck shaft angle and the femoral offset (P=0.024)
Adult* ; Arm ; Femur ; Femur Neck ; Head ; Humans ; Individuality

BACKGROUND: Flecainide is an antiarrhythmic agent that is used primarily in the treatment of cardiac arrhythmias. Some evidences also suggest that flecainide can participate in alveolar fluid clearance and inflammatory responses. This experiment was aimed to evaluate the effects of flecainide on sepsis induced acute lung injury in a rat model. METHODS: Rats were treated with subcutaneous infusion of saline or flecainide (0.1 or 0.2 mg/kg/hr) by a mini-osmotic pump. Subcutaneous infusion was started 3 hours before and continued until 8 hours after intraperitoneal injection of saline or endotoxin. Animals were sacrificed for analyses of severity of acute lung injury with wet to dry (W/D) ratio and lung injury score (LIS) in lung and inflammatory responses with level of leukocyte, polymorphonuclear neutrophils (PMNs) and inteleukin-8 (IL-8) in bronchoalveolar lavages fluid (BALF). RESULTS: Flecainide markedly improved dose dependently sepsis induced acute lung injury as analysed by W/D ratio (from 2.24 ± 0.11 to 1.76 ± 0.09, p < 0.05) and LIS (from 3 to 1, p < 0.05), and inflammatory response as determined by leukocyte (from 443 ± 127 to 229 ± 95, p < 0.05), PMNs (from 41.43 ± 17.63 to 2.43 ± 2.61, p < 0.05) and IL-8 (from 95.00 ± 15.28 to 40.00 ± 10.21, p < 0.05) in BALF. CONCLUSIONS: Flecanide improve sepsis induced acute lung injury in rats by controlling inflammatory responses.
Acute Lung Injury* ; Animals ; Arrhythmias, Cardiac ; Bronchoalveolar Lavage ; Flecainide* ; Infusions, Subcutaneous ; Injections, Intraperitoneal ; Interleukin-8 ; Leukocytes ; Lung ; Lung Injury ; Models, Animal ; Neutrophils ; Rats ; Sepsis*

Spindle cell hemangioendothelioma was first described in 1986 by Weiss and Enzinger as a low grade angiosarcoma resr mbling a cavernous hemangioma and kaposis sarcoma. Recently, it is suggested to be non neoplastic lesion or reactive process arising from pre-existing vascular mal- formation. We report a case of spindle cell hemangioendothelioma in a 9-month-old boy. He had multiple, variable sized, colorful, cutaneous or subcutaneous nodules on the forearm and hand. The tumor first appeared on the forearm as erythematous patches at birth and grew rapidly with- in 3 months. Histopatholgical findings showed that the lesion was composed of thin walled cavernous spaces mixed with spindle cells and occasional epithelioid endothelial cells containing intracytoplasmic vacuole. Most af the endothelial cells lining the cavernous spaces and intracytoplasmic lumina, were positive for factor VIII associated antigen. But the spindle cells were negative. Atypical vascular structures resembling arteriovenous shunts were noted around the tumor suggesting a reactive proliferation due to disturbance of local blood flow. Several turnors were excised. No recurrence has been recognized in the one year- follow-up period.
Endothelial Cells ; Factor VIII ; Follow-Up Studies ; Forearm ; Hand ; Hemangioendothelioma* ; Hemangioma, Cavernous ; Hemangiosarcoma ; Humans ; Infant ; Male ; Parturition ; Recurrence ; Sarcoma, Kaposi ; Vacuoles

BACKGROUND: The bcl-2 is a newly known oncogene involved in tumorigenisis by blocking apoptosis or programmed cell death. Overexpression of bcl-2 protein has been detected in a variety of human malignancies. However, recent studies of the expression of bcl-2 protein in human melanoma and melanocytic nevus have been controversial. OBJECTIVE: The purpose of this study was to examine whether there are any differences in the expression of bcl-2 protein between melanocytic nevus and rnalignant melanoma. METHODS: Immunohistochemical analysis of bcl-2 protein expression was performed on the formalin-fixed, paraffin-embedded tissue sections of 22 melanocytic nevus and 29 malignant melanomas (20 primary and 9 metastatic) using anti bcl-2 monoclonal antibody with an avidin-biotin peroxidase complex procedure. RESULTS: The results were as follows. 1. The positive rate for bcl-2 protein was observed in 95.4% (21/22) of melanocytic nevus and 95.0% (19/20) of primary malignant melanomas. Therefore, there was no significant difference between the two groups in the positive rate for bcl-2 prtoein. 2. The percentage of stained cells and the staining intensity of bcl-2 protein were significantly increased in melanocytic nevus compared to malignant melanoma (p<0.05). 3. The positive rate for bcl-2 expression of metastatic malignant melanoma [44.4% (4/9)] was significantly decreased compared to that of primary malignant melanoma [95.0%(19/20) ] (p<0. 05). But, there was no significant difference betweeen tumor thickness and histological type of malignant, melanoma in the expression of bcl-2 protein. 4. In melanocytic nevus, immunoreactivity of bcl-2 protein gradually diminished or even disappeared towards the deep dermis. CONCLUSION: the bcl-2 expression was decreased in malignant melanoma compared to melanocytic nevus. It. suggests that the loss of bcl-2 expression may play a significant role in the progression and metastasis of malignant melanoma.
Apoptosis ; Cell Death ; Dermis ; Humans ; Melanoma* ; Neoplasm Metastasis ; Nevus, Pigmented* ; Oncogenes ; Peroxidase

The pseudolymphoma syndrome that may mimic malignant lymphoma clinically and histopathologically has been described as a hypersensitivity reaction to anticonvulsant drugs. It consists of the triad of a fever, generalized rash and lyrnphadenopathy. In some cases, varying degrees of malaise, hepatosplenornegaly, abnormal liver function tests, arthralgias, eosinophilia and blood dyscriasias may also be present. We report a case of pseudolymphoma syndrome due to carbamazepine in a 47-year-old man. He had a diffuse edematous and erythematous patches, papules and vesicles associated with high fever, hepatosplenomegaly and lymphadenopathy. The histopathologic finding slowed a dense atypical mononuclear cell infiltrate of the upper dermis associated with Pautriers microabscess like structures and severe dermal edema. Laboratory findings revealed leukocytosis and an abnormal liver function test. Three weeks after the withdrawal of carbamazepine and treatment with oral prednisolone, his skin lesion and general condition markedly improved.
Anticonvulsants ; Arthralgia ; Carbamazepine* ; Dermis ; Edema ; Eosinophilia ; Exanthema ; Fever ; Humans ; Hypersensitivity ; Leukocytosis ; Liver Function Tests ; Lymphatic Diseases ; Lymphoma ; Middle Aged ; Prednisolone ; Pseudolymphoma* ; Skin

Mitral valvular prolapse (MVP) in dogs is characterized by myxomatous valvular degeneration, which is caused by abnormal valvular thickening and incomplete coaptation of the mitral valve leading to mitral regurgitation. Mitral regurgitation causes left atrial and left ventricular enlargement. Pathogenesis of the disease is unknown, although some studies have suggested the involvement of endothelin and systemic connective tissue diseases. Mitral valvular prolapse in dogs commonly occurs in aged small dog breeds, including Malteses and Shih Zhus. This case study investigated the clinical features of an affected Maltese family and performed pedigree analysis. To the best of the authors' knowledge, this is the first report of putative familial mitral valve prolapse and regurgitation in Maltese dogs. All family members in this study showed degenerative valvular changes and echocardiographic features of mitral valvular prolapse. Although disease progression differed, all dogs progressed to advanced heart failure stage within 2-3 years after diagnosis. Therefore, this is the first study to identify putative familial mitral valve prolapse in Maltese dogs. This finding suggests strong genetic etiology involved in the development of degenerative mitral valve disease in Maltese dogs. Furthermore, this finding could be a valuable resource for the identification of gene mutations in dogs with familial mitral valvular prolapse.
Animals ; Connective Tissue Diseases ; Diagnosis ; Disease Progression ; Dogs* ; Echocardiography ; Endothelins ; Heart Failure ; Humans ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Prolapse* ; Pedigree ; Prolapse

No abstract available.
Barium* ; Diagnosis* ; Enema* ; Hirschsprung Disease*