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Hypochondroplasia is an autosomal dominant skeletal dysplasia expressing postnatal onset of short stature with mild rhizomelic shortening of the limbs. This manifestation leads to restricted prenatal diagnosis of the disorder. We report here on a sporadic case of a hypochondroplastic baby, whose prenatal sonographic measurements were serially recorded from 19 weeks of gestation. Mild shortening of the limbs became manifest after 26 weeks of gestation. Biparietal diameter was within the normal range throughout gestation. Both parents were of average stature. A tentative diagnosis of a nonlethal short-limb skeletal dysplasia was made. At birth, the clinical manifestations of the neonate were not characteristic, but the radiographic features raised the possibility of hypochondroplasia. Molecular analyses revealed a C to G mutation at nucleotide 1659 of the fibroblast growth factor receptor 3 (FGFR3) gene, a common mutation in hypochondroplasia. Copyright © 2004 John Wiley & Sons, Ltd.  相似文献   
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Histological and electron microscopical studies were made on the fusion reaction between homogeneic stolons of the colonial ascidians Perophora japonica Oka and P. sagamiensis Tokioka. When two stolons make a tip-to-side contact, the epidermal cells of the lateral portion of one stolon become thick and form a protrusion. In the fusion reaction of P. japonica, epidermal cells of each stolon at the contact area extend cell processes immediately after contact. After an occurrence of degenerated epidermal cells, blood interchange between the two stolons is initiated by a partial elimination of the epidermal cells. Even after the establishment of blood interchange, the cuticlelike structure is often observed to remain at the contact area. In the fusion reaction of P. japonica, many amoebocytes are attached to the epidermal cells until the complete elimination of epidermal cells. In the fusion reaction of P. sagamiensis, many lymphocytes as well as amoebocytes are attached to the epidermal cells at the contact area.Contribution No. 461 of the Shimoda Marine Research Center  相似文献   
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We used dibasic acid esters as the absorbent for a volatile organic compounds (VOC) recovery and reuse system geared mainly toward VOCs from painting and presswork. The system consists of three elements: absorption using dibasic acid esters, separation by vacuum spray flash method, and recovery by condensation. The concentration of both ethyl acetate (EtAc) and isopropyl alcohol (IPA) decreased around 90% at room temperature. Vacuum spray flash was found as a useful process for cost-effective and energy-effective VOC removal system. The difference in the dissolubility parameters (Hansen constant) between dibasic acid esters and VOCs and the boiling point of VOCs was the main contributor to the separation behavior.  相似文献   
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The requirement to assess the management effectiveness (ME) in protected areas (PAs) is increasing around the world to help improve management and accountability. An evaluation of ME for Khojir National Park (KNP), one of the Iran’s oldest PAs, was conducted using a multi-method approach that consisted of structured interviews, open interviews, and site visits. This was the first ME evaluation in Iran. The structured interview was based on the management effectiveness tracking tool methodology. KNP received an average score of 43 %, which is lower than the global average, illustrating that its general management was in the low-intermediate level. The indices of legal status, resource inventory, planning for land and water use, regulations, and objectives received the highest average scores, whereas education and awareness, community co-management, regular work plan, boundary demarcation, visitor facilities, budget sources, staff training, protection systems, and management plan received the lowest ones. The management system of KNP was generally established, but many problems of the management still need to be resolved. To improve ME, some countermeasures should be taken, such as increasing funding, strengthening capacity building, planning, and adaptive management, and implementing community participation.  相似文献   
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In this study, an automatic sampling device and an analysis device have been developed for the measurement of low-volatile organic chlorine (LVOCl) in flue gas. The concentrations of dioxins (polychlorinated dibenzo-p-dioxins, polychlorinated dibenzofurans, and dioxin-like polychlorinated biphenyls) have been estimated by online measurements of LVOCl at a municipal solid waste incinerator (MSWI) using these devices. The LVOCl concentration at the outlet of the selective catalyst reactor (SCR) of the MSWI increased momentarily up to 95 μg Cl m−3 during the startup period of the MSWI; subsequently, it gradually decreased to less than 1.0 μg Cl m−3 after 50 h from the start of waste feeding. The concentration of toxic equivalent quantity (TEQ)-Dioxins at the SCR outlet had a linear positive relationship with the LVOCl concentrations. Moreover, the level of TEQ-Dioxins concentration can be estimated by using this relationship with LVOCl. From our results, since the LVOCl concentrations in a flue gas can thus be automatically analyzed every hour by online measurements, the operators of an MSWI would be able to monitor approximate TEQ-Dioxin emissions on a daily basis.  相似文献   
18.

Purpose

We evaluate malathion toxicity to Japanese medaka (Oryzias latipes) juveniles by using a mass spectrometry combined with gas chromatography (GC/MS) metabolomics approach.

Methods

Medaka were exposed to low (L) and high (H) concentrations (nominally 20 and 2,000 ??g/L, respectively) of water-borne malathion. Metabolites were extracted from the fish, derivatized, and analyzed by GC/MS. Identified metabolites were subjected to one-way analysis of variance and principal component analysis (PCA). We examined the variations in the amounts of the metabolites during the exposure period.

Results and discussion

At 24 h, control, L, and H groups were separated along PC1, suggesting that the effects of malathion depended on exposure concentration. The PCA results at 96 h suggest that the metabolite profiles variations of the L and H groups differed, and thus that the effects of malathion in groups differed. At 24 h, the amounts of amino acids in both exposed groups were lower than the control group amounts, perhaps owing to accelerated protein synthesis. At 96 h, the amounts of almost all the amino acids increased in the L group but decreased in the H group relative to the control group amounts, suggesting the proteolysis occurred in the L group while protein synthesis continued in the H group, that the high malathion exposure affected the fish. In addition, at 96 h, gluconeogenesis may have been induced in the L group but not in H group.

Conclusions

Malathion exposure may have altered the balance between protein synthesis and degradation and induced gluconeogenesis in medaka. Our results suggest that metabolomics will be useful for comprehensive evaluation of toxicity.  相似文献   
19.
Following the oil spill accident of the Solar I tanker in 2006 off the coast of Guimaras Island in the Philippines, polycyclic aromatic hydrocarbons (PAHs) and alkylated PAHs in some aquatic organisms were investigated at Luzaran in Guimaras and Taklong Islands, which were heavily polluted with spilled oil, immediately and 1 month after the accident. The concentrations of total PAHs were 11.9–52.3 ng/g dry weight in fish. Meanwhile, total PAH concentrations in shellfish were 38.0–3,102 ng/g dry weight in Luzaran and 128–236 ng/g dry weight in Taklong. Pyrene, phenanthrene, and fluoranthene were dominant in most fish and chrysene in all shellfish. Significantly higher concentrations of all alkylated homologs were detected in shellfish than in fish. These differences had two possible causes, that is, the differences between fish and shellfish could be attributed to the uptake routes and/or their metabolizing abilities.  相似文献   
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