The causative pathomechanism of sporadic amyotrophic lateral sclerosis (ALS) is not clearly understood. Using microarray technology combined with laser- captured microdissection, gene expression profiles of degenerati...The causative pathomechanism of sporadic amyotrophic lateral sclerosis (ALS) is not clearly understood. Using microarray technology combined with laser- captured microdissection, gene expression profiles of degenerating spinal motor neurons isolated from autopsied patients with sporadic ALS were examined. Gene expression was quantitatively assessed by real- time reverse transcription polymerase chain reaction and in situ hybridization. Spinal motor neurons showed a distinct gene expression profile from the whole spinal ventral horn. Three percent of genes examined were downregulated, and 1% were upregulated in motor neurons. Downregulated genes included those associated with cytoskeleton/ axonal transport, transcription, and cell surface antigens/receptors, such as dynactin, microtubule- associated proteins, and early growth response 3 (EGR3). In contrast, cell death- associated genes were mostly upregulated. Promoters for cell death pathway, death receptor 5, cyclins A1 and C, and caspases- 1, - 3, and - 9, were upregulated, whereas cell death inhibitors, acetyl- CoA transporter, and NF- κ B were also upregulated. Moreover, neuroprotective neurotrophic factors such as ciliary neurotrophic factor (CNTF), Hepatocyte growth factor (HGF), and glial cell line- derived neurotrophic factor were upregulated. Inflammation- related genes, such as those belonging to the cytokine family, were not, however, significantly upregulated in either motor neurons or ventral horns. The motor neuron- specific gene expression profile in sporadic ALS can provide direct information on the genes leading to neurodegeneration and neuronal death and are helpful for developing new therapeutic strategies.展开更多
The purpose of this study was to evaluate the perioperative complications in fetoscopy-guided laser therapy in feto-fetal transfusion syndrome. Study design: Details of feto-fetal transfusion syndrome cases who were d...The purpose of this study was to evaluate the perioperative complications in fetoscopy-guided laser therapy in feto-fetal transfusion syndrome. Study design: Details of feto-fetal transfusion syndrome cases who were delivered between 1999 and 2004 in a single center were reviewed retrospectively. Results: One hundred seventy-five procedures were performed percutaneously with the use of local anesthesia. Survival at 6 months of at least 1, 1, and 2 babies was 73% , 38% , and 35% , respectively. Placental abruption and miscarriage was diagnosed in 3 and 12 cases, respectively. Premature rupture of membranes occurred in 49 cases (28% ) and including 12, 29, and 46 cases that occurred before 24, 28, and 34 weeks of gestation, respectively. The entry of the trocar was transpla- cental in 48 cases (27% ), but it was not associated with miscarriage (P = .26), premature rupture of membranes (P = .58), or placental abruption (P = .37). Conclusion: Fetoscopic laser treatment of feto-fetal transfusion syndrome can be performed percutaneously with local anesthesia without significant maternal morbidity. Transplacental entry was not associated with premature rupture of membranes or miscarriage.展开更多
Background: We report on a patient with Graves’disease with radiation retinop athy caused by low-dose irradiation and antithyroid drug-induced antineutrophi l cytoplasmic antibody (ANCA)-positive vasculitis. Case: A ...Background: We report on a patient with Graves’disease with radiation retinop athy caused by low-dose irradiation and antithyroid drug-induced antineutrophi l cytoplasmic antibody (ANCA)-positive vasculitis. Case: A 38-year-old woman with Graves’disease presented with bilateral blurred vision, microaneurysms, te langiectasia, and macular edema. The patientwas examined by ophthalmoscopy and f luorescein angiography, and radiation retinopathywas diagnosed. Observations: Th e patient had been treated with low-dose irradiation for her Graves’ophthalmop athy a few years earlier. She also had ANCA-positive vasculitis induced by the antithyroid drug (propylthiouracil, PTU) that had been prescribed for her at tha t time. Because of multiple avascular areas on both retinas, she was treated by intensive retinal photocoagulation to control progressive retinopathy. Conclusio ns: The radiation doses used to treat Graves’disease ophthalmopathy are low. Ne vertheless, there is still a risk of radiation retinopathy developing in patient s with PTU-induced ANCA-positive vasculitis.展开更多
文摘The causative pathomechanism of sporadic amyotrophic lateral sclerosis (ALS) is not clearly understood. Using microarray technology combined with laser- captured microdissection, gene expression profiles of degenerating spinal motor neurons isolated from autopsied patients with sporadic ALS were examined. Gene expression was quantitatively assessed by real- time reverse transcription polymerase chain reaction and in situ hybridization. Spinal motor neurons showed a distinct gene expression profile from the whole spinal ventral horn. Three percent of genes examined were downregulated, and 1% were upregulated in motor neurons. Downregulated genes included those associated with cytoskeleton/ axonal transport, transcription, and cell surface antigens/receptors, such as dynactin, microtubule- associated proteins, and early growth response 3 (EGR3). In contrast, cell death- associated genes were mostly upregulated. Promoters for cell death pathway, death receptor 5, cyclins A1 and C, and caspases- 1, - 3, and - 9, were upregulated, whereas cell death inhibitors, acetyl- CoA transporter, and NF- κ B were also upregulated. Moreover, neuroprotective neurotrophic factors such as ciliary neurotrophic factor (CNTF), Hepatocyte growth factor (HGF), and glial cell line- derived neurotrophic factor were upregulated. Inflammation- related genes, such as those belonging to the cytokine family, were not, however, significantly upregulated in either motor neurons or ventral horns. The motor neuron- specific gene expression profile in sporadic ALS can provide direct information on the genes leading to neurodegeneration and neuronal death and are helpful for developing new therapeutic strategies.
文摘The purpose of this study was to evaluate the perioperative complications in fetoscopy-guided laser therapy in feto-fetal transfusion syndrome. Study design: Details of feto-fetal transfusion syndrome cases who were delivered between 1999 and 2004 in a single center were reviewed retrospectively. Results: One hundred seventy-five procedures were performed percutaneously with the use of local anesthesia. Survival at 6 months of at least 1, 1, and 2 babies was 73% , 38% , and 35% , respectively. Placental abruption and miscarriage was diagnosed in 3 and 12 cases, respectively. Premature rupture of membranes occurred in 49 cases (28% ) and including 12, 29, and 46 cases that occurred before 24, 28, and 34 weeks of gestation, respectively. The entry of the trocar was transpla- cental in 48 cases (27% ), but it was not associated with miscarriage (P = .26), premature rupture of membranes (P = .58), or placental abruption (P = .37). Conclusion: Fetoscopic laser treatment of feto-fetal transfusion syndrome can be performed percutaneously with local anesthesia without significant maternal morbidity. Transplacental entry was not associated with premature rupture of membranes or miscarriage.
文摘Background: We report on a patient with Graves’disease with radiation retinop athy caused by low-dose irradiation and antithyroid drug-induced antineutrophi l cytoplasmic antibody (ANCA)-positive vasculitis. Case: A 38-year-old woman with Graves’disease presented with bilateral blurred vision, microaneurysms, te langiectasia, and macular edema. The patientwas examined by ophthalmoscopy and f luorescein angiography, and radiation retinopathywas diagnosed. Observations: Th e patient had been treated with low-dose irradiation for her Graves’ophthalmop athy a few years earlier. She also had ANCA-positive vasculitis induced by the antithyroid drug (propylthiouracil, PTU) that had been prescribed for her at tha t time. Because of multiple avascular areas on both retinas, she was treated by intensive retinal photocoagulation to control progressive retinopathy. Conclusio ns: The radiation doses used to treat Graves’disease ophthalmopathy are low. Ne vertheless, there is still a risk of radiation retinopathy developing in patient s with PTU-induced ANCA-positive vasculitis.
