ABSTRACT
A malignant mesenchymoma is a rare soft tissue neoplasm that show at least two types of malignant mesenchymal differentiation, in addition to poorly differentiated fibrosarcomatous elements. They grow rapidly, recur frequently, and can be found in a wide variety of location, with most occurring in the extremities. A primary malignant mesenchymoma of the heart is extremely rare, with only 20 cases having been reported worldwide in the English literature up to 2002. We experienced a case of primary cardiac malignant mesenchymoma in a 59 year old female patient, who was admitted due to exertional dyspnea. This case is reported, with a review of the relevant literature.
Subject(s)
Female , Humans , Middle Aged , Dyspnea , Extremities , Heart Neoplasms , Heart , Mesenchymoma , Soft Tissue NeoplasmsABSTRACT
We analyzed the comparative amounts of granulocyte-colony stimulating factor (G-CSFr) and granulocyte macrophage CSF (GM-CSFr) receptors expressed on neutrophils and monocytes in measles patients to investigate the role of these CSFrs in the development of leukopenia including neutropenia and monocytopenia in measles. EDTA-anticoagulated peripheral blood of 19 measles patients, 10 children with other infections showing leukopenia and 16 children with normal complete blood cell counts (CBC)s were analyzed using flow cytometry and QuantiBRITE. The leukocyte (5260 +/- 2030/uL vs. 9900 + 2680/uL, p=0.000), neutrophil (2580 +/- 960/uL vs. 4250 +/- 2750/uL, p=0.024) and the lymphocyte counts of measles patients (1810 +/- 1430/uL vs. 4530 +/- 3450/uL, p= 0.006) were lower than in the normal controls. The neutrophils of measles patients expressed similar amounts of G- CSFr (1858 +/- 355) as normal children (1764 +/- 477, p= 0.564) and leukopenic patients (1773 +/- 673, p=0.713), but lower levels of GM-CSFr (535 +/- 118) than normal children (957 +/- 344, p=0.000) and leukopenic patients (832 +/- 294, p=0.002). The monocytes of measles patients expressed similar amounts of G-CSFr (916 +/- 336) and GM-CSFr (3718 +/- 906) as normal children (1013 +/- 391 and 4125 (2645, p > 0.05) but less than leukopenic patients (1454 +/- 398 and 5388 +/- 806, p > 0.05). The neutrophil and monocyte counts of measles patients did not correlate with the amount of G-CSFr or GM-CSFr expressed on neutrophils or monocytes (p > 0.05), but in the normal children, the monocyte count correlated with the levels of GM-CSFr on monocytes (r=0.951, p=0.049). In conclusion, neutropenia is one of the more important characteristics of measles patients, which could be due to the decreased GM-CSFr expression on neutrophils. However, the monocytopenia found in measles patients is not due to the decreased expression of CSFr on the monocytes.
Subject(s)
Humans , Leukocyte Count , Measles/blood , Monocytes/chemistry , Neutropenia/etiology , Neutrophils/chemistry , Receptors, Granulocyte Colony-Stimulating Factor/blood , Receptors, Granulocyte-Macrophage Colony-Stimulating Factor/bloodABSTRACT
PURPOSE: Without a specific diagnostic method, the clinical diagnostic criteria for atypical Kawasaki disease is known as the presence of coronary artery changes in a patient who did not meet the already estabilished clinical criteria. With this criteria, we are put into dilemma because atypical Kawasaki disease should exhibit coronary artery disease. So, we demonstrated the rate of coronary artery involvement in atypical Kawasaki disease by another diagnostic criteria. METHODS: This diagnostic criteria for atypical Kawasaki disease is "the clinical symptoms which fulfilled four out of six diagnostic criteria below of typical Kawasaki disease with or without the presence of coronary artery changes, and were inconsistent under a through clinical investigation with any other illness similar to Kawasaki disease." RESULTS: The incidence of coronary artery involvement in atypical Kawasaki disease during the acute phase (analysis of 30 cases who were admitted before the 15th day of illness), on the 30th day of illness (26 cases) and on the 60th day of illness(26 cases) were 20.0% (dilatation : 13.3%, aneurysm : 6.7%), 19.2% (dilatation : 19.2%, aneurysm : 0.0%), 7.7%(dilatation : 7.7%, aneurysm : 0.0%), respectively. CONCLUSOIN: Kawasaki disease may occur in atypical type, ranging over many severe involvements of vital organs including the coronary artery. Up to the present, there are no infallible diagnostic tests for Kawasaki disease and it is advisable to deemphasize the rigid adherence to the current already proposed diagnostic criteria for Kawasaki disease.
Subject(s)
Humans , Aneurysm , Coronary Artery Disease , Coronary Vessels , Diagnostic Tests, Routine , Incidence , Mucocutaneous Lymph Node SyndromeABSTRACT
Administration of G-CSF may not always respond in rise of neutrophil counts in different patient population. In order to understand a possible inter-relationship between the G-CSF and GM-CSF induced leukocyte responses and expression levels of receptors for G-CSF (G-CSFr) and GM-CSF (GM-CSFr), the levels of each receptor and CSF were measured in patients with basophilia (8), eosinophilia (14) and bacterial infection showing neutrophilia (12) in comparison with normal healthy adults (12) and children (14). G-CSFr was expressed in neutrophils in the largest amount followed by monocytes, but GM-CSFr was expressed more in monocytes than neutrophils. Lymphocytes and basophils did not express G-CSFr or GM-CSFr. The amount of GM-CSFr in neutrophils was present less in patients with infection than normal control (P = 0.031). The neutrophils expressed more G-CSFr than GM-CSFr. The quantity of G-CSFr in eosinophil showed marked interval change, higher in acute stage. The plasma concentrations of G-CSF in patients with infection were much higher than normal adults or children (117.95 +/- 181.16 pg/ml, P < 0.05). Binding assay with excess amount of CSFs could discriminate the patient who did not show any response to G-CSF or GM-CSF administration. After incubation with excess CSFs, more receptors were blocked in children than in adults (G-CSF P = 0.024, GM-CSF P = 0.006). These results indicate that the amount of CSFr in leukocyte varies in different types of leukocyte, and changes according to the patients' condition even in the same type of leukocyte, and the CSFrs of children bind to CSFs more than those of adults.
Subject(s)
Adult , Child , Humans , Bacterial Infections , Basophils/chemistry , Breast Neoplasms , Colony-Stimulating Factors/blood , Eosinophilia , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Leukocyte Disorders , Monocytes/chemistry , Neoplasms , Neutrophils/chemistry , Receptors, Colony-Stimulating Factor/analysis , Receptors, Granulocyte Colony-Stimulating Factor/analysis , Receptors, Granulocyte-Macrophage Colony-Stimulating Factor/analysisABSTRACT
Scimitar syndrome is a rare congenital anomaly that is characterized by hypoplasia of the right lung and the right pulmonary artery with anomalous pulmonary venous drainage to the inferior vena cava. The scimitar vein is usually visible on chest radiographs, but may be obscured by the heart. It is essential for surgical correction to establish the point of drainage of the anomalous vein and associated anomalies. There are recent reports of familial total anomalous pulmonary venous return suggesting heritable forms of this anomaly. Although genetic factors are believed to have important roles in congenital heart disease, few genes involved in heart development have been located. We report a case of familial chromosome 9 inversion with Scimitar syndrome in an offspring who presented with dextrocardia. Evaluation with magnetic resonance cineangiograph imaging demonstrated an anomalous pulmonary vein draining into the inferior vena cava above the diaphragm and hypoplasia of the right lung and the right pulmonary artery. Chromsome analysis showed pericentric inversion of chromosome 9, inv 9 (p13, q21), in the patient and his mother as well. A brief review of the related literature is also included.
Subject(s)
Humans , Chromosomes, Human, Pair 9 , Dextrocardia , Diaphragm , Drainage , Heart , Heart Defects, Congenital , Lung , Mothers , Pulmonary Artery , Pulmonary Veins , Radiography, Thoracic , Scimitar Syndrome , Veins , Vena Cava, InferiorABSTRACT
PURPOSE: Doppler echocardiography provides a noninvasive technique for the evaluation of left ventricular diastolic function. This study was performed to define the normal pattern of left ventricular diastolic filling by real-time Doppler flow mapping, and to determine how this pattern changes with aging in normal children. METHODS: The changes in diastolic function in 89 normal subjects less than 15 years old were assessed through the analysis of transmitral Doppler flow pattern. Diastolic time intervals, Doppler flow velocities and area fractions in early and late diastole were measured from mitral valve Doppler tracing. The changes in each variable with aging were determined. RESULTS: From the diastolic time intervals, acceleration and deceleration time increased significantly with aging. The percentage of isovolumic relaxation time to cycle length decreased with aging, but there were no significant differences in the percentage of acceleration and deceleration time to cycle length between each age group. The Doppler area of early diastolic flow, E and 0.33 area, significantly increased with aging than that of late diastolic flow, A area. The E and A area fractions to total area were significantly different in the age group of less than 2 months old compared with those of other age groups. The ratios of early to late diastolic flow velocities and early to late area fractions were increased with aging. CONCLUSION: Although many factors affect transmitral Doppler flow pattern, it is important to know the changes of left ventricular diastolic filling with aging to accurately evaluate diastolic dysfunction in children. Further investigation of work in large populations is needed to provide reference values of children with aging.
Subject(s)
Adolescent , Child , Humans , Infant , Acceleration , Aging , Deceleration , Diastole , Echocardiography, Doppler , Mitral Valve , Reference Values , RelaxationABSTRACT
We experienced a case of persistent left superior vena cava draining into the common atrium in a fetus of 26-year-old primigravida. Persistent left superior vena cava is derived from the left cardinal vein. This anomalous vessel usually drains into the right atrium via the coronary sinus and has no physiologic significance. Much less frequently, the left superior vena cava drains directly into the left atrium and it is associated with complex heart anomalies such as atrial septal defect, absence of coronary sinus, double outlet right ventricle, transposition of great arteries and asplenia syndrome. Fetal echocardiography showed an abnormal vessel draining into common atrium in a fetus with complex heart anomalies. It had a different direction from right superior vena cava or pulmonary veins in its traveling. We report a case with autosy findings.
Subject(s)
Adult , Humans , Coronary Sinus , Double Outlet Right Ventricle , Echocardiography , Fetus , Heart , Heart Atria , Heart Septal Defects, Atrial , Heterotaxy Syndrome , Pulmonary Veins , Transposition of Great Vessels , Veins , Vena Cava, SuperiorABSTRACT
Epidermal nevus syndrome is a rare disease which includes disorders of bone, central nervous system, eye, kidney, vasculature, body symmetry, skin, and rarely, hypophosphatemic rickets which have been observed in association with epidermal nevi. We experienced a case of epidermal nevus syndrome. He presented with epidermal nevi and bilateral abdominal wall hernias at birth and developed right hemiparesis, mental retardation, and multiple fractures due to hypophosphatemic rickets. He died of pneumonia at the age of 6.
Subject(s)
Abdominal Wall , Central Nervous System , Hernia , Intellectual Disability , Kidney , Nevus , Paresis , Parturition , Pneumonia , Rare Diseases , Rickets, Hypophosphatemic , SkinABSTRACT
Epidermal nevus syndrome is a rare disease which includes disorders of bone, central nervous system, eye, kidney, vasculature, body symmetry, skin, and rarely, hypophosphatemic rickets which have been observed in association with epidermal nevi. We experienced a case of epidermal nevus syndrome. He presented with epidermal nevi and bilateral abdominal wall hernias at birth and developed right hemiparesis, mental retardation, and multiple fractures due to hypophosphatemic rickets. He died of pneumonia at the age of 6.
Subject(s)
Abdominal Wall , Central Nervous System , Hernia , Intellectual Disability , Kidney , Nevus , Paresis , Parturition , Pneumonia , Rare Diseases , Rickets, Hypophosphatemic , SkinABSTRACT
PURPOSE: This study was designed to investigate whether valproic acid(VPA) induces Reye's syndrome by analyzing blood chemistry, carnitine(CARN) concentration, and the morphologic changes of hepatocytes. The changes of GFAP immunoreactivity of astrocytes were examined in rats with Reye's syndrome induced by VPA. And the effects of CARN supplementation on Reye's syndrome were also investigated. METHODS: Ten rats were assigned to each of the following 3 groups : control group(0.5ml of normal saline), VPA group(250mg/kg of VPA), and VPA plus CARN group(250mg/kg of VPA and 100mg/kg of CARN). Each dose was injected intraperitoneally every 12 hours for 7 days. All rats fasted overnight following the final injection. RESULTS: In the VPA group, the levels of glucose and ketones decreased and the levels of ammonia, SGOT and lactates increased as compared with the control group. Lower levels of free CARN and higher acyl/free CARN ratios, indicating secondary CARN deficiency, were observed, and the appearance of transformed mitochondria of hepatocytes provided further evidence of mitochondrial metabolic dysfunction in Reye's syndrome. The GFAP immunoreactivity and the area percent of GFAP-positive astrocytes in the cingulum decreased in VPA group. In the VPA plus CARN group, there were no significant differences with the control group in blood chemistry, acyl/free CARN ratios and morphology of hepatocytes. However, the GFAP immunoreativity and the percent of GFAP-positive astrocytes in the cingulum decreased as much as in the VPA group. CONCLUSION: It is suggested that CARN supplementation may prevent abnormal findings of blood chemistry, CARN deficiency and the morphologic changes of liver mitochondria due to VPA, but CARN may have little effect on GFAP immunoreactivity of astrocytes in rats with Reye's syndrome induced by VPA.
Subject(s)
Animals , Rats , Ammonia , Aspartate Aminotransferases , Astrocytes , Carnitine , Chemistry , Glucose , Hepatocytes , Ketones , Lactates , Mitochondria , Mitochondria, Liver , Reye Syndrome , Valproic AcidABSTRACT
Visualization of coronary collaterals in coronary arteriography performed in vivo constitutes an important finding, and particularly in the presence of coronary artery disease, it allows observations related to the hemodynamic consequences of the disease process. Undoubtedly, the presence of collateral flow indicates that a compensatory mechanism has developed to ameliorate the detrimental effect of blood flow cessation due to obstruction of the arterial pathways. By definition, intracoronary collaterals are connections between branches of the same coronary artery. In general, in a coronary arteriogram obtained in vivo, their incidence is lower than that of intercoronary collaterals. We experienced a case of intracollateral circulation, seen extending from the aneurysm through the first segment filled with the right ventricular branch like vessels (OC, Seg 1. CL, intra. Seg 1-2. ANl, Seg 1. CL, inter. Seg 4-8.). Intercollaterals originated from the distal circumflex branch of the left coronary artery, passing through a posterior descending branch and later filled the right ventricular branch of the right coronary artery (OC, Seg 15. LSmd, Seg 6. ANm, Seg 6-7. CL, inter. Seg 15-4.) in a 9-year-old male with Kawasaki disease.
Subject(s)
Child , Humans , Male , Aneurysm , Angiography , Constriction, Pathologic , Coronary Artery Disease , Coronary Vessels , Hemodynamics , Incidence , Mucocutaneous Lymph Node SyndromeABSTRACT
A rare but serious complication of tuberculosis in a child occurs when the primary infection is progressively destructive. It is difiicult to differentiate from other bacterial pneumonias. We experienced a case of tuberculous pneumonia in a 73-days-old male who presented with cough, fever and progressive dyspnea. Chest X-ray shows diffuse pneumonic consolidation on right upper lung field, Mantoux test was positive and his father was diagnosed with active pulmonary tuberculosis 2 months ago. We started antituberculous drugs: INH, RFP, PZA, SM and prednisolone. At 12 weeks after therapy, chest CT and percutaneous aspiration lung biopsy was performed due to worsening of X-ray findings. The diagnosis of tuberculous pneumonia was confirmed. He was treated with INH, RFP, PZA for 52 weeks. Short-term corticosteroid therapy and SM or KM was also used. The pneumonic consolidation was totally cleared and healed with fibrosis.
Subject(s)
Child , Humans , Male , Biopsy , Cough , Diagnosis , Dyspnea , Fathers , Fever , Fibrosis , Lung , Pneumonia , Pneumonia, Bacterial , Prednisolone , Thorax , Tomography, X-Ray Computed , Tuberculosis , Tuberculosis, PulmonaryABSTRACT
Pseudomembranous colitis may result from abnormal growth of toxigenic strains of Clostridium difficile associated with antibiotic-induced alteration of the colonic microbiologic flora. C. difficile, a spore forming gram positive obligate anaerobic bacillus, is part of the normal flora and known to be the major cause of pseudomembranous colitis. We experienced a case of pseudomembranous colitis in a 14-month-old male presented with bloody mucoid diarrhea and fever who had received broad spectrum antibiotics for the treatment of pneumonia. This case was diagnosed with sigmoidoscopy and confirmed by isolation of the organism on culture with selective media TCCFA. All antibiotics were discontinued and vancomycin was administrated orally. He showed a good response.
Subject(s)
Humans , Infant , Male , Anti-Bacterial Agents , Bacillus , Clostridioides difficile , Clostridium , Colon , Diarrhea , Enterocolitis, Pseudomembranous , Fever , Pneumonia , Sigmoidoscopy , Spores , VancomycinABSTRACT
Pseudomembranous colitis may result from abnormal growth of toxigenic strains of Clostridium difficile associated with antibiotic-induced alteration of the colonic microbiologic flora. C. difficile, a spore forming gram positive obligate anaerobic bacillus, is part of the normal flora and known to be the major cause of pseudomembranous colitis. We experienced a case of pseudomembranous colitis in a 14-month-old male presented with bloody mucoid diarrhea and fever who had received broad spectrum antibiotics for the treatment of pneumonia. This case was diagnosed with sigmoidoscopy and confirmed by isolation of the organism on culture with selective media TCCFA. All antibiotics were discontinued and vancomycin was administrated orally. He showed a good response.