No abstract available.
Neoplasm Metastasis* ; Pancreatic Neoplasms* ; Scalp* ; Thorax*

BACKGROUND AND OBJECTIVES: It has been reported that various inflammatory and immune reactions are involved in the development and progression of atherosclerosis. We tried to investigate whether the TNF receptor superfamilies are involved in the development and progression of atherosclerosis. MATERIALS AND METHOD: Thirteen carotid atheroma specimens(frozen sections : 10 cases, paraffin section : 5 cases) were obtained from the patients who underwent carotid endarterectomy at Samsung Medical Center and one normal aortic tissue was obtained from a transplantation donor in brain death. In the carotid endarterectomy specimens and a normal aortic tissue , the expressions of R110(TR1), 139(TR2) and DR3(TR3), members of the TNF receptor superfamilies were evaluated by immunohistochemical staining with monoclonal antibodies. Simultaneously, we evaluated the expressions of foam cells, smooth muscle cells, T-lymphocytes and B-lymphocytes. RESULTS: Immunohistochemical analysis identified a strong expressions of foam cells and smooth muscle cells in all atheroma. But, the expression of T-lymphocytes was minimal and that of B-lymphocytes was rare. The expression of DR3(TR3) was seen in all atheroma as strongly positive. The expression of 139(TR2) was observed well in frozen sections, but not in paraffin sections. Whereas, that of R110(TR1) was observed in paraffin sections as weakly positive, but not in frozen section. The areas where the TNF receptor superfamilies were expressed correlated to the area of foam cell presence. The expression of DR3 also correlated with expression of smooth muscle cells. In normal aortic tissue, the expression of inflammatory cells or TNF receptor superfamilies was not observed except smooth muscle cells which were observed in normal artery. CONCLUSION: Foam cells and smooth muscle cells were abundantly present in atheroma. The TNF receptor superfamilies are expressed in the atheroma and the region of expression was coincident with the presence of foam cells.
Antibodies, Monoclonal ; Arteries ; Atherosclerosis ; B-Lymphocytes ; Brain Death ; Endarterectomy, Carotid ; Foam Cells ; Frozen Sections ; Humans ; Myocytes, Smooth Muscle ; Paraffin ; Plaque, Atherosclerotic* ; Receptors, Tumor Necrosis Factor* ; T-Lymphocytes ; Tissue Donors ; Tumor Necrosis Factor-alpha*

True mixed liposarcomas are extremely rare tumors. We report here on a case of mixed liposarcoma that was composed of well differentiated and pleomorphic liposarcoma. A 76-year-old man presented to us with a mass in his left upper arm. This lesion had been there for twenty years, it was recently growing rapidly and had doubled in size during the recent 2 months. The MR image showed a mass composed of a fat component and a soft tissue component with necrosis. The old fat component was revealed as well differentiated liposarcoma, and the recent growing soft tissue component was revealed as pleomorphic liposarcoma. The two components showed different immunohistochemical results for MDM2.
Aged ; Arm ; Humans ; Liposarcoma* ; Necrosis

BACKGROUND: Protamine sulfate (PS), used to neutralize the effect of heparin, is often associated with systemic hypotension. The present study was aimed to investigate the protective effects of CaCl2 on adverse hemodynamic reaction to PS. METHODS: Forty-six dogs of three groups were studied. Animals were randomly allocated to receive either; saline 10 ml (controls, n = 26), CaCl2 5 mg/kg (n = 10) or 10 mg/kg (n = 10), added to PS 3 mg/kg given intravenously 5 min after heparin (300 IU/kg, iv). Mean aortic pressure (MAP), mean pulmonary arterial pressure (MPAP), left ventricular end-diastolic pressure (LVEDP), LVdP/dtmax, and -LVdP/dtmin, and pulmonary artery (cardiac output) and left circumflex blood flow (LCX flow) were recorded up to 15 min after PS. RESULTS: PS caused significant reductions in MAP (-49%), cardiac index (CI, -66%), systemic vascular resistance index (SVRI, -22%), LVEDP (-67%), LVdP/dtmax (-36%), and LVdP/dtmin (-55%), but increased MPAP (39%) and PVRI (3.8 fold), which all increases and reductions peaked 1-3 min after PS injection in the control group. The addition of CaCl2 to PS significantly attenuated reductions of MAP (-35 and -26% in CaCl2 5 and 10 mg/kg treated dogs, respectively), CI (-34 and -37%), LVdP/dtmax (-14, -11% ), and -LVdP/dtmin (-34, -21%), and increases in PVRI (1.8 and 2.4 fold). However, both doses of CaCl2 increased MPAP (48, 82%), but not SVRI (-28, -21%) or LVEDP (-73, -75%). LCX flow increased similarly in all groups (75-137%). CaCl2 5 and 10 mg/kg iv increased plasma Ca2+ by 0.23 and 0.36 mM, respectively. CONCLUSIONS: These results suggest that the simultaneous administration of CaCl2 attenuates the adverse hemodynamic effects of PS, used to reverse heparin anticoagulation in the dog.
Animals ; Arterial Pressure ; Calcium Chloride* ; Calcium* ; Dogs* ; Hemodynamics* ; Heparin ; Hypotension ; Plasma ; Protamines* ; Pulmonary Artery ; Vascular Resistance

Chronic granulomatous disease(CGD) is a genetically inherited disorder caused by the failure of phagocytic cells to produce superoxide. It is characterized by frequent and uncontrollable infection which often lead to death in early childhood. The first clinical signs may be confined to skin and manifest themselves as abscesses, pyoderma, eczema or draining sinuses. The disease was first reported in 1957 and thereafter the biomolecular mechanism has been found. The first report in Korea was an autopsy case in 1979 and since then there have been a few case reports. This disease is diagnosed by symptoms, such as high fever, cervical lymphadenopathy, skin nodule, lung field infiltration, periumbilical abscess, liver abscess, pyoderma and pericordal abscess. The pathogen here were catalase producing bacteria and fungi. However, we have experienced a case of CGD, in which symptoms were perianal, scrotal abscesses and lung field nodular infiltration. In this case the pathogen was found as a catalase negative Enterococcus. (J Korean Pediatr Soc 2000;43-846-850)
Abscess ; Autopsy ; Bacteria ; Catalase ; Eczema ; Enterococcus ; Fever ; Fungi ; Granulomatous Disease, Chronic* ; Korea ; Liver Abscess ; Lung ; Lymphatic Diseases ; Phagocytes ; Pyoderma ; Skin ; Superoxides

It is well known that both acute glomerulonephritis and rheumatic fever are a common sequelae of group A streptococcal infection. However, their simultaneous occurrence is uncommon. Only a few cases have been reported because they are different in epidemiologic, clinical, serologic and immunopathogenic characteristics. We experienced a 10-year-old boy who presented the manifestation of acute rheumatic fever and acute poststreptococcal glomerulonephritis. We report this case with brief review of literature.
Child ; Glomerulonephritis* ; Humans ; Male ; Rheumatic Fever* ; Streptococcal Infections

BACKGROUND: The pathogenesis of psoriasis may involve the interleukin (IL)-23 and Th17-mediated immune responses. Th17 cells secret IL-17 and IL-22, which mediates dermal inflammation and acanthosis. OBJECTIVE: As inhibitor of nuclear factor kappaB kinase-alpha (IKKalpha) has been previously identified as a primary regulator of keratinocyte differentiation and proliferation, we proposed that IL-17 and IL-22 might affect keratinocyte differentiation by changing the expression of IKKalpha. METHODS: We employed HaCaT cells maintained culture medium at a low calcium concentration (0.06 mM) and induced differentiation by switching to the high concentration (2.8 mM) media with IL-17 or IL-22, then compared the IKKalpha expression and the cell cycle. We employed reconstituted human epidermal skin (Neoderm) and mice ears for the in vivo studies. RESULTS: Elevated calcium concentration induced IKKalpha expression and terminal differentiation with cell cycle arrest in HaCaT cell cultures. Moreover, IL-17 and IL-22 treatment also induced IKKalpha in HaCaT cells and reconstituted human epidermis. IKKalpha induction was also noted, following the injection of IL-17 and IL-22 into mice ears. CONCLUSION: Although the induction of IKKalpha was accompanied by keratinocyte differentiation, IL-17 and IL-22 did not affect calcium-mediated differentiation or the cell cycle. Rather, IL-17 and IL-22 appear to contribute to the inflammation occurring via the induction of IKKalpha from keratinocytes or skin layers.
Animals ; Calcium ; Cell Culture Techniques ; Cell Cycle ; Cell Cycle Checkpoints ; Cell Differentiation ; Ear ; Epidermis ; Humans ; I-kappa B Kinase ; Inflammation ; Interleukin-17 ; Interleukins ; Keratinocytes ; Mice ; Psoriasis ; Skin ; Th17 Cells

Pseudohypoparathyroidism(PHP) is a genetic disorder characterized by target cell resistance to the effect of parathyroid hormone(PTH). The disorder is classified into type I a, I b, I c and II depending on the phenotype and biochemical findings. In type I a, urinary cyclic AMP and urinary phosphate excretion are not increased after PTH stimulation because of deficient G unit activity in target cells. Deficiency of the G unit is a generalized cellular defect and accounts for the association of other endocrine disorders with type I a PHP. Type I b PHP shows resistance to PTH but not to other hormones and normal phenotypic appearance. In type I c PHP affected children have defect in catalytic unit of adenylate cyclase and in addition to resistance to PTH, resistance to the metabolic effects of TSH, gonadotropins, and glucagon may be detected. Typical appearance of Albright's hereditary osteodystrophy is common in PHP type Ia and Ic. In type II PHP, urinary cyclic AMP response is generated but this does not lead to phosphaturia. We experienced two patients with PHP. One is a 11-year-old girl diagnosed type I a PHP and the other is a 11-year-old boy suspected type I b PHP. They visited emergency room because of tetanic movement. Both patients had no previous history of tetany and showed low serum calcium level, high phosphorus level and high PTH level. The girl had typical features of Albright's hereditary osteodystrophy such as round face, short neck, obese feature, brachydactyly and mental retardation but didn't have basal ganglia calcification on brain CT. The boy showed normal appearance and no mental retardation.
Adenylyl Cyclases ; Basal Ganglia ; Brachydactyly ; Brain ; Calcium ; Child ; Cyclic AMP ; Emergency Service, Hospital ; Female ; Glucagon ; Gonadotropins ; Humans ; Hypophosphatemia, Familial ; Intellectual Disability ; Male ; Neck ; Phenotype ; Phosphorus ; Pseudohypoparathyroidism* ; Tetany

OBJECTIVES: We aimed to identify the causative mutation for siblings in a Korean family with nonsyndromic hearing loss (HL) and enlarged vestibular aqueduct (EVA). The siblings were a 19-year-old female with bilateral profound HL and an 11-year-old male with bilateral moderately severe HL. METHODS: We extracted genomic DNA from blood samples of the siblings with HL, their parents, and 100 controls. We performed mutation analysis for SLC26A4 using direct sequencing. RESULTS: The two siblings were compound heterozygotes with the novel mutation p.I713LfsX8 and the previously described mutation p.H723R. Their parents had heterozygous mono-allelic mutations. Father had p.I713LfsX8 mutation as heterozygous, and mother had p.H723R mutation as heterozygous. However, novel mutation p.I713LfsX8 was not detected in 100 unrelated controls. CONCLUSION: Both mutations identified in this study were located in the sulfate transporter and anti-sigma factor antagonist domain, the core region for membrane targeting of SulP/SLC26 anion transporters, which strongly suggests that failure in membrane trafficking by SLC26A4 is a direct cause of HL in this family. Our study could therefore provide a foundation for further investigations elucidating the SLC26A4-related mechanisms of HL.
Child ; DNA ; Fathers ; Female ; Frameshift Mutation* ; Hearing Loss* ; Hearing* ; Heterozygote ; Humans ; Male ; Membranes ; Mothers ; Parents ; Siblings ; Vestibular Aqueduct* ; Young Adult

Brief ischemic episodes that induce myocardial and coronary endothelial dysfunction may alter the responses to inotropic drugs. To determine the effects of inotropic drugs in stunned myocardium, the coronary blood flow (CBF), myocardial oxygen consumption (MVO2), and regional mechanical function in response to intracoronary dobutamine, epinephrine, amrinone, and calcium chloride (CaCl2) were measured before (normal) and 30 min after a 15-min-period occlusion of the left anterior descending artery (stunned) in an open-chest canine model. Percent segment shortening (%SS) and post-systolic shortening (%PSS) were determined. Myocardial extraction of oxygen (EO2) and lactate (E(lac)) was calculated. The inotropic drugs increased %SS, CBF, and MVO2 in normal myocardium. Epinephrine and amrinone decreased, while dobutamine and CaCl2 did not affect EO2. The ischemia and reperfusion itself significantly reduced %SS and E(lac), and increased %PSS. In stunned myocardium, the responses to inotropic drugs were not significantly altered, except that they progressively reduced %PSS and epinephrine did not affect EO2. These findings indicate that a brief episode of ischemia does not affect the mechanical and metabolic coronary flow responses to inotropic drugs, although it abolishes direct vasodilator responses to epinephrine.
Amrinone/administration and dosage ; Animals ; Calcium Chloride/administration and dosage ; Cardiotonic Agents/*administration and dosage ; Comparative Study ; Dobutamine/administration and dosage ; Dogs ; Dose-Response Relationship, Drug ; Epinephrine/administration and dosage ; Female ; Male ; Myocardial Contraction/*drug effects ; Myocardial Stunning/*drug therapy/etiology/*physiopathology ; Oxidation-Reduction/drug effects ; Oxygen Consumption/*drug effects ; Reperfusion Injury/complications/*drug therapy/*physiopathology ; Treatment Outcome