Leiomyosarcoma is an uncommon soft tissue sarcoma of mesenchymal cell origin, which shows smooth muscle differentiation. Leiomyosarcoma is seldom found in the pediatric population, and accounts for fewer than 2% of all soft tissue sarcomas. Leiomyosarcoma of the chest wall is extremely rare in children. We report here a case of an 8-year-old boy with a primary leiomyosarcoma that was incidentally found as a rib mass. The patient underwent a complete resection for a suspected osteochondroma diagnosed by a three-dimensional chest computed tomography examination. Pathological findings of the mass revealed intersecting fascicles of spindle cells showing cigar-shaped nuclei, inconspicuous nuclear pleomorphism and occasional mitotic figures in the background of a suspected osteochondroma of the rib. This report documents the first description of a leiomyosarcoma possibly arising in an osteochondroma of the rib in a child.
Child ; Humans ; Leiomyosarcoma ; Muscle, Smooth ; Osteochondroma ; Ribs ; Sarcoma ; Thoracic Wall ; Thorax

PURPOSE: The objective of this study was to observe the neurodevelopmental outcomes of the surviving very low birth weight infants (VLBWIs) and to identify the perinatal risk factors having influences on to poor neurodevelopmental outcomes . METHODS: The VLBWIs weighing 500 to 1,499 g at birth who had survived to discharge from one NICU during about a 2 year period were followed-up and assessed with using the Baley Scales of Infant Development-Second Edition (BSID-II) test and neurologic examinations when the infants corrected age was between 12 and 24 months. Developmental delay was defined as a MDI less than 70 or a PDI less than 70. The birthweight specific rates of developmental delay and cerebral palsy were examined. The perinatal data were retrospectively collected from the medical records to identify peinatal risk factors that had an influence on poor neurologic outcomes. RESULTS: Thirty three (42.9%) of the 77 VLBWIs were assessed with the BSID-II and neurologic examination, when their corrected age was between 12 and 24 months. The rate of developmental delay and cerebral palsy in the assessed infants was 15.2% and 21.2%, respectively. Extremely low birth weight infants (ELBWIs) had high rates of developmental delay (30.8%) and cerebral palsys (30.8%). Maternal old age (>35 years, odds ratio=18.0, 95% CI, 1.2-262.7, P=0.035) and periventricular leukomalacia (PVL, odds ratio=12.6, 95% CI, 1.1-148.1, P=0.044) were independently associated with developmental delay and cerebral palsy, respectively. CONCLUSION: Significant poor neurodevelopmental outcome for the VLBW infants needs a more extended follow-up study for development, and especially for the ELBWIs.
Cerebral Palsy ; Follow-Up Studies ; Humans ; Infant* ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Leukomalacia, Periventricular ; Medical Records ; Neurologic Examination ; Parturition ; Retrospective Studies ; Risk Factors ; Weights and Measures

BACKGROUND: In perinatal hypoxic-ischemic encephalopathy (HIE), cerebral blood flow is impaired and the activity of nitric oxide systhase (NOS) is markedly increased. For the association with the development of a stroke, the endothelial NOS (eNOS) polymorphisms are well-known. METHODS: Three clinically relevant polymorphisms of the eNOS gene were determined in 37 term/near-term infants with perinatal HIE (HIE group) and 54 normal term newborn infants without any perinatal problems (control group) using a polymerase chain reaction with or without restriction fragment enzyme digestion. The differences in the genotype, allele, and haplotype frequencies were evaluated between the groups. RESULTS: The analysis of the allele frequencies showed that the G allele of Glu298Asp was more frequent in the HIE group than in the controls. The comparisons between the controls and each subgroups with complications that occurred with HIE showed that the TC genotype and C allele of T(-786)C were more common in patients with persistent pulmonary hypertension of the newborn (PPHN) than in the controls. The frequency of the A b T haplotype was lower in the HIE patients than in the controls. CONCLUSIONS: The G allele of Glu298Asp was associated with perinatal HIE, while the TC genotype and C allele of T(-786)C were associated with PPHN.
Alleles ; Digestion ; Gene Frequency ; Genotype ; Haplotypes ; Humans ; Hypertension, Pulmonary ; Hypoxia-Ischemia, Brain ; Infant ; Infant, Newborn ; Nitric Oxide ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Stroke

PURPOSE: The objective of this study is to observe high-resolution computed tomography (HRCT) findings of lung parenchyme in very low birth weight (VLBW) infants between the corrected age of 38-42 weeks who were treated with oxygen after birth, and to compare them to the clinical severity of bronchopulmonary dysplasia (BPD). METHODS: The lungs of fourty-four VLBW infants with gestational ages of less than 32 weeks and birth weights of less than 1,500 g who were treated with oxygen after birth were examined using HRCT taken when the corrected age was between 38-42 weeks. Common findings among the infants and the frequency of their occurrences were noted. Total CT scores obtained by the summation of air trapping and actelectasis scores and the ratio of bronchus-to-pulmonary artery diameter were used to quantitatively evaluate HRCT findings and correlate them with the clinical severity of BPD as defined by Jobe-Bancalari diagnostic criteria. RESULTS: 1) The most common findings in HRCT images of the lungs were air trapping (56%), atelectasis (70.5%), linear opacity (77%), and distortion of the bronchopulmonary bundle (65.9%). These findings were more commonly observed in infants with BPD in a mixed pattern than those without (P<0.05). However, abnormal findings were also found in HRCT images of some infants without BPD. In infants with BPD, air trapping, atelectasis and total CT scores were higher than those without BPD. Also infants with BPD had a lower bronchus-to-pulmonary artery diameter than those without BPD (P<0.05). 2) The total CT scores (r=0.799, P<0.0001) and the ratio of bronchus- to-pulmonary artery diameter (r=0.576, P<0.0001) showed a linear correlation with the clinical severity of BPD. CONCLUSION: HRCT findings in VLBW infants between the corrected age of 38-42 weeks who had been treated with oxygen after birth are useful in revealing pathologic changes in the lung parenchyme and show a good correlation with the clinical severity of BPD.
Arteries ; Birth Weight ; Bronchopulmonary Dysplasia ; Gestational Age ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Lung* ; Oxygen Inhalation Therapy ; Oxygen* ; Parturition ; Pulmonary Atelectasis

PURPOSE: We intended to observe cell death and apoptotic changes in neurons in organotypic hippocampal slice cultures following oxygen-glucose deprivation (OGD), using propidium iodide (PI) uptake, Fluoro-Jade (FJ) staining, TUNEL staining and immunofluorescent staining for caspase-3. METHODS: The hippocampus of 7-day-old rats was cut into 350 micrometer slices. The slices were cultured for 10 d (date in vitro, DIV 10) and and exposed to OGD for 60 min at DIV 10. They were then incubated for reperfusion under normoxic conditions for an additional 48 h. Fluorescence of PI uptake was observed at predetermined intervals, and the cell death percentage was recorded. At 24 h following OGD, the slices were Cryo-cut into 15 micrometer thicknesses, and Fluoro-Jade staining, TUNEL staining, and immunofluorescence staining for caspase-3 were performed. RESULTS: 1) PI uptake was restricted to the pyramidal cell layer and DG in the slices after OGD. The fluorescent intensities of PI increased from 6 to 48 h during the reperfusion stage. The cell death percentage significantly increased time-dependently in CA1 and DG following OGD (P< 0.05). 2) At 24 h after OGD, many FJ positive cells were detected in CA1 and DG. Some neurons had distinct nuclei and processes while others had fragmented nuclei and disrupted processes in CA1. TUNEL and immunofluorescent staining for caspase-3 showed increased expression of TUNEL labeling and caspase-3 in CA1 and DG at 24 h after OGD. CONCLUSION: The numerous dead cells in the slice cultures after OGD tended to display apoptotic changes mediated by the activation of caspase-3.
Animals ; Anoxia ; Apoptosis ; Brain ; Caspase 3 ; Cell Death ; Fluoresceins ; Fluorescence ; Fluorescent Antibody Technique ; Hippocampus ; In Situ Nick-End Labeling ; Ischemia ; Neurons ; Propidium ; Pyramidal Cells ; Rats ; Reperfusion

No abstract available.