Hemangiopericytoma is a rare vascular tumor arising from pericytes. The tumor usually develops in the skin or subcutaneous tissue, particularly in the extremities.4 pulmonary origin of hemangiopericytoma is known to be quite rare. It has the potential to become a highly malignant lesion, so wide excision is the treatment of choice. We present a case of primary malignant hemangiopericytoma of the lung and discuss the clinical symptoms, diagnosis, therapy and prognosis within the context of a brief review.
Diagnosis ; Hemangiopericytoma* ; Lung* ; Pericytes ; Prognosis ; Skin ; Subcutaneous Tissue

The authors presented a 17 months old female with malignant osteopetrosis, who was admitted to the Pediatric Department of Kyung Hee University Hospital on May, 1981 because of epistaxis and growth retardation. Diagnosis was made by typical clinical manifestations, hematologic and radiologic findings, such as frontal bossing, opened anterior fontanel, exophalmoses, strabismus, optic atropy, marked hepatosplenomegaly, severe anemia, thrombocytopenia, reticulocytosis and typical mask sign and sun burst sign at the skull X-ray, wide dense ribs with fracture at the vertebrae, clubbings at the distal parts of the femurs and radii and at the proximal parts of the tibias and humerii transverse bands at the metaphyses, and finally increased density of the entire skeleton on the bone scanning and marked decrease uptake of 99mTc on the bone marrow scanning. Prednisolone was administrated orally, resulted in gradual improvement of hematologic abnormalities to the normal range, associated wited with increased cellularity of the bone marrow and increased uptake of 99mTc and decreased spleen size on the bone marrow scanning after one month of therapy.
Anemia ; Bone Marrow ; Cranial Fontanelles ; Diagnosis ; Epistaxis ; Female ; Femur ; Humans ; Infant ; Masks ; Osteopetrosis* ; Prednisolone ; Reference Values ; Reticulocytosis ; Ribs ; Skeleton ; Skull ; Solar System ; Spine ; Spleen ; Strabismus ; Thrombocytopenia ; Tibia

The authors presented a 17 months old female with malignant osteopetrosis, who was admitted to the Pediatric Department of Kyung Hee University Hospital on May, 1981 because of epistaxis and growth retardation. Diagnosis was made by typical clinical manifestations, hematologic and radiologic findings, such as frontal bossing, opened anterior fontanel, exophalmoses, strabismus, optic atropy, marked hepatosplenomegaly, severe anemia, thrombocytopenia, reticulocytosis and typical mask sign and sun burst sign at the skull X-ray, wide dense ribs with fracture at the vertebrae, clubbings at the distal parts of the femurs and radii and at the proximal parts of the tibias and humerii transverse bands at the metaphyses, and finally increased density of the entire skeleton on the bone scanning and marked decrease uptake of 99mTc on the bone marrow scanning. Prednisolone was administrated orally, resulted in gradual improvement of hematologic abnormalities to the normal range, associated wited with increased cellularity of the bone marrow and increased uptake of 99mTc and decreased spleen size on the bone marrow scanning after one month of therapy.
Anemia ; Bone Marrow ; Cranial Fontanelles ; Diagnosis ; Epistaxis ; Female ; Femur ; Humans ; Infant ; Masks ; Osteopetrosis* ; Prednisolone ; Reference Values ; Reticulocytosis ; Ribs ; Skeleton ; Skull ; Solar System ; Spine ; Spleen ; Strabismus ; Thrombocytopenia ; Tibia

BACKGROUND: Smoking is a risk factor for idiopathic pulmonary fibrosis (IPF), but the mechanism of the association remains obscure. There is evidence demonstrating that plasminogen activator inhibitor-1 (PAI-1) is involved in the progression of pulmonary fibrosis. This study was to determine whether the administration of small interfering RNA (siRNA) targeting PAI-1 or PAI-1 inhibitor to the cigarette smoking extract (CSE)-exposed rat alveolar type II epithelial cells (ATII cells) limits the epithelial-mesenchymal transition (EMT). METHODS: ATII cells were isolated from lung of SD-rat using percoll gradient method and cultured with 5% CSE. The EMT was determined from the ATII cells by measuring the real-time RT PCR and western blotting after the PAI-1 siRNA transfection to the cells and after administration of tiplaxtinin, an inhibitor of PAI-1. The effect of PAI-1 inhibitor was also evaluated in the bleomycin-induced rats. RESULTS: PAI-1 was overexpressed in the smoking exposed ATII cells and was directly associated with EMT. The EMT from the ATII cells was suppressed by PAI-1 siRNA transfection or administration of tiplaxtinin. Signaling pathways for EMT by smoking extract were through the phosphorylation of SMAD2 and ERK1/2, and finally Snail expression. Tiplaxtinin also suppressed the pulmonary fibrosis and PAI-1 expression in the bleomycin-induced rats. CONCLUSION: Our data shows that CSE induces rat ATII cells to undergo EMT by PAI-1 via SMAD2-ERK1/2-Snail activation. This suppression of EMT by PAI-1 siRNA transfection or PAI-1 inhibitor in primary type II alveolar epithelial cells might be involved in the attenuation of bleomycin-induced pulmonary fibrosis in rats.
Animals ; Blotting, Western ; Epithelial Cells ; Epithelial-Mesenchymal Transition ; Idiopathic Pulmonary Fibrosis ; Indoleacetic Acids ; Lung ; Phosphorylation ; Plasminogen ; Plasminogen Activator Inhibitor 1 ; Plasminogen Activators ; Polymerase Chain Reaction ; Povidone ; Pulmonary Fibrosis ; Rats ; Risk Factors ; RNA, Small Interfering ; Silicon Dioxide ; Smoke ; Smoking ; Snails ; Transfection

BACKGROUND: Obstructive sleep apnea syndrome (OSAS) affects systemic blood pressure and cardiac function. The development of cardiovascular dysfunction including the changes of systemic blood pressure and cardiac rhythm, suggests that recurrent hypoxia and arousals from sleep may increase a sympathetic nervous system activity. Continuous positive airway pressure(CPAP) therapy has been found to be an effective treatment of OSAS. However, only a few studies have investigated the cardiovascular and sympathetic effects of CPAP therapy. We evaluated influences of nasal CPAP therapy on the cardiovascular system and the sympathetic activity in patients with OSAS. METHODS: Thirteen patients with OSAS underwent CPAP therapy and were monitored using polysomnography, blood pressure, heart rate, presence of arrhythmia and the concentration of plasma catecholamines, before and with CPAP therapy. RESULTS: The apnea-hypopnea index (AHI) was significantly decreased (p<0.01) and the lowest arterial oxygen saturation level was elevated significantly after applying CPAP (p<0.01). Systolic blood pressure tended to decrease after CPAP but without statistical significance. Heart rates during sleep were not significantly different after CPAP. However, the frequency and number of types of arrhythmia decreased and sinus bradytachyarrhythmia disappeared after CPAP. Although there was no significantly decreased after CPAP (p<0.05). CONCLUSION: CPAP therapy decreased the apnea-hypopnea index, hypoxic episodes and plasma norepinephrine concentration. In addition, it decreased the incidence of arrhythmia and tended to decrease the systemic blood pressure. These results indicate that CPAP may play an important role in the prevention of cardiovascular complications in patients with OSAS.
Anoxia ; Arousal ; Arrhythmias, Cardiac ; Blood Pressure* ; Cardiovascular System ; Catecholamines* ; Heart Rate ; Hemodynamics ; Humans ; Incidence ; Norepinephrine ; Oxygen ; Plasma ; Polysomnography ; Sleep Apnea, Obstructive* ; Sympathetic Nervous System

Extramammary Paget's disease of the vulva is a rare lesion that accounts for 1~2% of vulvar neoplasms. Paget's disease often has a microscopic extension beyond the gross lesion and shows a multifocal distribution. Positive resection margin is common. We applied 5-FU cream on the lesion who had a positive resection margin. There was no residual lesion on multiple punch biopsies after 5-FU cream treatment three months later. And there has been no evidence of disease recurrence in the 12-month follow-up period. We experienced a case of effective treatment with 5-FU cream in microinvasive Paget's disease of the vulva with positive resection margin. We present it with a brief review of literatures.
Biopsy ; Fluorouracil ; Follow-Up Studies ; Recurrence ; Vulva ; Vulvar Neoplasms

We report a case of spontaneous renal rupture in a 63 year-old woman as a complication of diabetes mellitus. She experienced a sudden onset of severe pain and bulging mass on the left flank. She had been suffered from diabetes mellitus for 30 years without any specific treatment, and she had no history of specific associated diseases or factors such as trauma, stone, tumor, hydronephrosis or vascular lesion of the kidney. Grossly this ruptured kidney showed a characteristic necrosis and infarcted lesions extended from papilla to cortex of the upper pole kidney and surrounding tissues of the kidney showed interstitial fibrosis, tubular atrophy and mononuclear cell infiltration. Microscopically the atherosclerotic medium-sized blood vessels and small vessels around the infarcted lesion also showed hyaline sclerosis. This pathologically defined spontaneous renal rupture is thought to be caused by focal renal infarction due to atherosclerosis or fat emboli-associated renovascular occlusion in diabetic patient. This report emphasizes the importance of diabetic nephropathy as a new cause of the spontaneous renal rupture in patients of diabetes mellitus.
Atherosclerosis ; Atrophy ; Blood Vessels ; Diabetes Mellitus* ; Diabetic Nephropathies ; Female ; Fibrosis ; Humans ; Hyalin ; Hydronephrosis ; Infarction ; Kidney* ; Middle Aged ; Necrosis ; Rupture ; Rupture, Spontaneous* ; Sclerosis

PURPOSE: The proportion of interventional catheterization in congenital heart disease is being increased in Korea, especially in Sejong Heart Institute, so we performed statistical analysis on the cardiac catheterization cases. METHODS: Total 233 cases of congenital heart disease confirmed by cardiac catheterization at the Sejong Heart Institute between Jaunary 1995 and June 1995 were analyzed retrospectively. RESULTS: 1) The distribution of age has become more younger than that of previous reports that is, the proportion of the infant less than 1 year old was 34.8%. 2) The proportion of male sex was equal to that of female. TOF and TGA were observed predominantly in male patients, and PDA and UVH revealed female preponderance. 3) The relative frequencies of each individual congenital heart disease in order of frequency were as follows; TOF 37.3%, PDA 13.3%, VSD 8.2% and UVH 7.7%. 4) Among the 145 (62%) cases of hemodynamic study pre-operation and post-operation cases were 85 and 60 cases, respectively. The cases of interventional catheterization were 84 (36%), and those of endomyocardial biopsy were 4 (2%). 5) The frequencies of each interventional catheterization of 84 cases in order of frequency were as follows: defect closure 28 cases, balloon valvuloplasty 20 cases, balloon angioplasty 17 cases, coil embolization 15 cases, and balloon atrial septostomy 4 cases. 6) There were 2 cases of mortality associated with cardiac catheterization. CONCLUSIONS: The proportion of interventional catheterization in congenital heart disease is being increased in the Sejong Heart Institute, and this trend will spread to the other cardiac center in Korea.
Angioplasty, Balloon ; Balloon Valvuloplasty ; Biopsy ; Cardiac Catheterization ; Cardiac Catheters ; Catheterization* ; Catheters* ; Embolization, Therapeutic ; Female ; Heart ; Heart Defects, Congenital* ; Hemodynamics ; Humans ; Infant ; Korea ; Male ; Mortality ; Retrospective Studies

BACKGROUND: We evaluated the clinical and radiologic results of impacted femoral neck fractures treated with multiple pinning and determined the influence of the progression of impaction at the fracture site on clinical outcome. METHODS: There were 34 patients with a mean age of 65.5 years. The mean follow-up period was 3.4 years. Progression of fracture site impaction was measured using an articulo-trochanteric distance index and the percentage decrease in the articulo-trochanteric distance index between follow-up intervals. The failure of treatment was clarified as non-union and avascular necrosis. Other characteristics of the patients, including mean waiting time for surgery, preoperative Singh index score, and body mass index, were also measured to evaluate the influence on the clinical outcome of surgery. RESULTS: There were 6 fractures which were not treated successfully (3 non-union, 8.8% and 3 avascular necrosis, 8.8%). The mean percentage decrease of the articulo-trochanteric distance index within the first 6 weeks after surgery was 4.5% in the successful group and 25.1% in the failure group (p < 0.001). There was also a significant mean percentage decrease in the articulo-trochanteric distance index between 6 weeks and 3 months (p < 0.001). CONCLUSIONS: Primary stabilization with Knowles pins for impacted femoral neck fractures had a reasonable clinical outcome with low morbidity. Despite a significant difference of a mean percentage decrease in the articulo-trochanteric distance index between the successful group and the failure group, we could not verify it as a risk factor for failure of treatment because the odds ratio was not statistically significant.
Adult ; Aged ; Aged, 80 and over ; Bone Nails ; Female ; Femoral Neck Fractures/complications/radiography/*surgery ; Femur Head Necrosis/etiology/radiography ; Follow-Up Studies ; *Fracture Fixation, Internal ; Fractures, Ununited/radiography ; Hip Joint/*pathology/radiography ; Humans ; Male ; Middle Aged ; Odds Ratio ; Treatment Outcome

BACKGROUND: We evaluated the clinical and radiologic results of impacted femoral neck fractures treated with multiple pinning and determined the influence of the progression of impaction at the fracture site on clinical outcome. METHODS: There were 34 patients with a mean age of 65.5 years. The mean follow-up period was 3.4 years. Progression of fracture site impaction was measured using an articulo-trochanteric distance index and the percentage decrease in the articulo-trochanteric distance index between follow-up intervals. The failure of treatment was clarified as non-union and avascular necrosis. Other characteristics of the patients, including mean waiting time for surgery, preoperative Singh index score, and body mass index, were also measured to evaluate the influence on the clinical outcome of surgery. RESULTS: There were 6 fractures which were not treated successfully (3 non-union, 8.8% and 3 avascular necrosis, 8.8%). The mean percentage decrease of the articulo-trochanteric distance index within the first 6 weeks after surgery was 4.5% in the successful group and 25.1% in the failure group (p < 0.001). There was also a significant mean percentage decrease in the articulo-trochanteric distance index between 6 weeks and 3 months (p < 0.001). CONCLUSIONS: Primary stabilization with Knowles pins for impacted femoral neck fractures had a reasonable clinical outcome with low morbidity. Despite a significant difference of a mean percentage decrease in the articulo-trochanteric distance index between the successful group and the failure group, we could not verify it as a risk factor for failure of treatment because the odds ratio was not statistically significant.
Adult ; Aged ; Aged, 80 and over ; Bone Nails ; Female ; Femoral Neck Fractures/complications/radiography/*surgery ; Femur Head Necrosis/etiology/radiography ; Follow-Up Studies ; *Fracture Fixation, Internal ; Fractures, Ununited/radiography ; Hip Joint/*pathology/radiography ; Humans ; Male ; Middle Aged ; Odds Ratio ; Treatment Outcome