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Despite advances in postnatal care, patients born with a congenital diaphragmatic hernia (CDH) suffer substantial morbidity and mortality. The present study was undertaken to determine the prognostic influence of prenatally-diagnosed liver herniation in the hemithorax in fetuses with CDH. The medical records of 48 patients evaluated for a prenatally-diagnosed left CDH were retrospectively reviewed. Patients were analysed according to the position of the liver by prenatal ultrasound; 32 fetuses had a major portion of the liver herniated into the left hemithorax ('liver up') and 16 had an intra-abdominal liver ('liver down'). Liver position was determined using colour-flow Doppler ultrasonography. There were two fetal deaths in the liver-up group and one in the liver-down group. The liver-up group more frequently required extracorporeal membrane oxygenation (ECMO) support (53 per cent) compared with the liver-down group (19 per cent). Postnatal survival was significantly less in the liver-up group (43 per cent) vs. the liver-down group (93 per cent). Fetuses with congenital diaphragmatic hernia and liver herniated into the hemithorax have a much worse prognosis than similarly afflicted fetuses without liver herniation. Prenatal ultrasonographic diagnosis of congenital diaphragmatic hernia allows for preparation for a critically ill newborn and aids in prenatal family counselling.  相似文献   
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The expression of cellular retinoic-acid-binding protein (CRABP) and cellular retinol-binding protein (CRBP), as well as their relationship to retinoic acid (RA) synthesis and degradation were examined in the developing mouse cerebellum and choroid plexus of the fourth ventricle. The choroid plexus, which expresses the RA-synthesizing retinaldehyde dehydrogenase RALDH-2, is likely to represent a diffusion source of RA for the closely apposed cerebellum, regulating its development. We found CRBP to be expressed in the choroid plexus and, in an in-vitro assay, addition of recombinant CRBP to RALDH-2 increased RA synthesis from retinaldehyde, with the amount of increase depending on the CRBP/retinaldehyde ratio. A technique that characterizes RA-binding proteins according to their isoelectric point showed both CRABP I and CRABP II to be present in the cerebellum and P19 cells, and only CRABP II to be present in the choroid plexus. With this technique, CRABP I could also be detected in the HL60 cell line. In addition to the two known acidic RA-binding proteins CRABP I and II, the cerebellum expressed a third RA-binding protein distinguishable by its neutral isoelectric point; the same binding protein was also detected in the olfactory bulb, kidney and testes. We used the RA-binding technique to follow the rate of elimination of bound RA from the cerebellum. A systemic injection of 0.3 micromols RA into postnatal day-1 mice was almost completely removed after 8 hours. These results suggest mechanisms by which the retinoid-binding protein may regulate the equilibrium of RA synthesis and catabolism in the cerebellum and choroid plexus.  相似文献   
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Homocystinuria is a rare inherited metabolic disease. Arterial and venous thromboembolic events represent frequent and life-threatening complications in homocystinuric patients. It has been suggested that mild homocysteinemia could be a risk factor for vascular disease. We have therefore measured total plasma homocysteine (HCy) concentrations by radioisotopic assay in 50 subjects with venous or arterial thrombosis and studied the relationship between HCy, coagulation and fibrinolytic parameters. Values were considered abnormal if they were higher than 2.7 standard deviations (SD) above the mean, i.e., 14.1 mmol/l. Thus, eighteen of the 50 patients with thrombosis were classified in the hyperhomocysteinemia group. Nine of these subjects had only this isolated risk factor. No correlations were found between HCy and antithrombin III, protein C, protein S and plasminogen levels, or plasma plasminogen activator inhibitor activity. Nevertheless, the correlation between tissue-plasminogen activator antigen and total plasma HCy was significant (r = 0.61, p < 0.001). Increased homocysteinemia seems to be a risk factor for thrombotic events especially knowing that HCy presents a direct cytotoxic effect. Vitamin therapy, already used in homozygote homocystinuric patients, might be beneficial in the prevention of thromboembolic disease in heterozygous patients.  相似文献   
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Primary brain tumors in two dogs were surgically removed followed by postoperative radiation therapy. The two tumors were confirmed histologically to be astrocytoma and meningioma, respectively. After the surgery, the neurological status of each dog improved dramatically and a total dose of 40 Gy was delivered to the surgical site to treat residual tumor tissue. Although the dog with astrocytoma died 6 months after surgery due to unknown causes, the dog with meningioma has lived for over 24 months with a degree of neurological disorder less severe than that before treatment. These results suggested the effectiveness of this type of therapy on brain tumor in dogs and therapeutic modality should be positively planned to treat canine brain tumors.  相似文献   
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Breast reconstruction has been the subject of many controversies. It now seems generally agreed however that the operation has no deleterious influence on the follow up of breast cancer patients and that it has favorable psychologic effects. Breast reconstruction can be performed either by using prostheses or analogous tissues. These two different modalities have each their own advantages and limitations. Deep knowledge of the techniques and their indications allows to select the most appropriate approach in the individual patient and, thus, to improve long term results.  相似文献   
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This study investigates the relation between performance on simple tasks dependent on proprioception, and performance of complex perceptual-motor skills in clumsy children and age-matched control children. One hundred and forty-six right-handed children aged between 5 and 8 years were tested on non-visual aiming, non-visual posture matching, the Kinaesthetic Sensitivity Test (KST), and the Movement Assessment Battery for Children (ABC). Half of the children had scores below the 15th centile on the Movement ABC and were classed into the developmental coordination disorder (clumsiness) group. Scores on the proprioceptive tasks were used to predict performance on complex tasks of the subscales of the Movement ABC (manual dexterity, ball skills, and balance). Specific relations were found between the proprioceptive tasks and the subscales of the Movement ABC, but the KST did not predict differences in motor skills, and no relation was found between tasks carried out without vision. Simple non-visual movement tasks do predict performance in more complex skilled tasks but this is affected by many task features rather than simply the reliance on proprioception for information about movement.  相似文献   
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