Objective： HPP （Hypophosphatasia） is a congenital disease characterized by a deficiency of tissue-specific ALP （alkaline phosphatase）, which causes the generation of abnormal bone and tooth tissue. The clinical m...Objective： HPP （Hypophosphatasia） is a congenital disease characterized by a deficiency of tissue-specific ALP （alkaline phosphatase）, which causes the generation of abnormal bone and tooth tissue. The clinical manifestations are variable, from neonatal forms with high mortality to milder adult forms with fractures and osteomalacia. The present study reports a clinical case of HPP, with emphasis on the physical characteristics and laboratory tests. The patient was attended at the medical genetics outpatient clinic of the Association of Parents and Friends of the Exceptional of Manaus （Associa？？o de Pais e Amigos dos Excepcionais-APAE/Manaus）. Methodology： For the case report, anamnesis, peripheral blood G-band karyotyping, Vitamin and ALP dosage and resonance examination were performed. Results： A.M.M.S, 9 years, non-consanguineous parents, no bone disease in the family, with one sister from the mother. Birth weight AGA without intercurrence. The patient presented a slight delay in psycho-motor development and was diagnosed with craniostenosis through CNS imaging examination. The physical examination, in addition to a short stature, showed dolichocephaly, frontal bossing, curved tibias and great dental compromise with many early cavities and loss of teeth. Laboratory evaluation： ALP dosage： 63/L （N = 69-325）; Vitamin B6 dosage： 27.4 （5.2-34.1）. The mother and the younger sister also had an ALP result below that expected for the age. Conclusions： The clinical phenotype and laboratory diagnosis of the patient were compatible with ICD Q78 HPP.展开更多
Although passive listening （i.e., hearing language） may occur in some EFL classes through oral reading, oral presentations, or other oral forms, listening instruction is a relatively neglected skill in EFL teaching,...Although passive listening （i.e., hearing language） may occur in some EFL classes through oral reading, oral presentations, or other oral forms, listening instruction is a relatively neglected skill in EFL teaching, and as a consequence, assessment of listening has received relatively limited coverage in the language testing literature. Interactive listening, based on the idea that listening is real-life communication, has been demonstrated to be a dimension of listening worth teaching. However, no successful classroom interactive listening teaching format has yet been reported, let alone assessed. Based on the models designed by previous researchers, an interactive listening teaching format was designed and trailed a university classroom intervention program in Beijing. In order to do this, it was necessary to construct an objective interactive listening assessment which could measure the listener＇s ability to： （a） negotiate meaning, and （b） to comprehend the speaker＇s messages （Macaro, 2003; Rost, 2002）. To verify its reliability and validity, a standard listening test, the types of negotiation, and selfassessment were also adopted. The results show that compared with traditional listening tests, this interactive listening assessment procedure evaluation of listeners＇ listening skills. provided a more comprehensive and objective展开更多
文摘Objective： HPP （Hypophosphatasia） is a congenital disease characterized by a deficiency of tissue-specific ALP （alkaline phosphatase）, which causes the generation of abnormal bone and tooth tissue. The clinical manifestations are variable, from neonatal forms with high mortality to milder adult forms with fractures and osteomalacia. The present study reports a clinical case of HPP, with emphasis on the physical characteristics and laboratory tests. The patient was attended at the medical genetics outpatient clinic of the Association of Parents and Friends of the Exceptional of Manaus （Associa？？o de Pais e Amigos dos Excepcionais-APAE/Manaus）. Methodology： For the case report, anamnesis, peripheral blood G-band karyotyping, Vitamin and ALP dosage and resonance examination were performed. Results： A.M.M.S, 9 years, non-consanguineous parents, no bone disease in the family, with one sister from the mother. Birth weight AGA without intercurrence. The patient presented a slight delay in psycho-motor development and was diagnosed with craniostenosis through CNS imaging examination. The physical examination, in addition to a short stature, showed dolichocephaly, frontal bossing, curved tibias and great dental compromise with many early cavities and loss of teeth. Laboratory evaluation： ALP dosage： 63/L （N = 69-325）; Vitamin B6 dosage： 27.4 （5.2-34.1）. The mother and the younger sister also had an ALP result below that expected for the age. Conclusions： The clinical phenotype and laboratory diagnosis of the patient were compatible with ICD Q78 HPP.
基金supported by"The Research on EFL Interactive Listening Teaching and Assessment"(Project No.: 2010JG34)
文摘Although passive listening （i.e., hearing language） may occur in some EFL classes through oral reading, oral presentations, or other oral forms, listening instruction is a relatively neglected skill in EFL teaching, and as a consequence, assessment of listening has received relatively limited coverage in the language testing literature. Interactive listening, based on the idea that listening is real-life communication, has been demonstrated to be a dimension of listening worth teaching. However, no successful classroom interactive listening teaching format has yet been reported, let alone assessed. Based on the models designed by previous researchers, an interactive listening teaching format was designed and trailed a university classroom intervention program in Beijing. In order to do this, it was necessary to construct an objective interactive listening assessment which could measure the listener＇s ability to： （a） negotiate meaning, and （b） to comprehend the speaker＇s messages （Macaro, 2003; Rost, 2002）. To verify its reliability and validity, a standard listening test, the types of negotiation, and selfassessment were also adopted. The results show that compared with traditional listening tests, this interactive listening assessment procedure evaluation of listeners＇ listening skills. provided a more comprehensive and objective
