Rapid Assessment Terhadap Kerusakan Bangunan Akibat Erupsi Merapi Tahun 2010

Erupsi Gunungapi Merapi di tahun 2010 memberikan dampak salah satunya adalah kerusakan bangunan. Penelitian ini bertujuan untuk memetakan dan menginventarisasi kerugian bangunan serta memberikan rekomendasi kebijakan rehabilitasi dan rekonstruksi tempat tinggal dan fasilitas permukiman, khususnya di wilayah Kabupaten Sleman. Metode penelitian yang digunakan adalah analisis penginderaan jauh dan sistem informasi geografis serta survey lapangan. Data dasar menggunakan Citra IKONOS, Citra ASTER dan Citra Geo eye-1. Hasil penelitian menunjukkan 3245 buah bangunan mengalami kerusakan berat hingga hancur, semuanya di Kecamatan Cangkringan. Wilayah ini direkomendasikan PVMBG menjadi Kawasan Rawan Bencana III Merapi. Penelitian merekomendasikan Pemerintah Daerah Kabupaten Sleman untuk melakukan relokasi warga dengan pendekatan persuasif dan sistematis berbasis sosial budaya, dengan sistem bedol dusun/kampung, penyediaan wilayah tujuan relokasi yang sesuai dengan wilayah asal, sistem tukar lahan, dan penyediaan fasilitas yang memadai. Rehabilitasi dan rekonstruksi juga perlu memperhatikan penyediaan fasilitas lingkungan permukiman antara lain fasilitas air bersih, fasilitas air limbah dan MCK, fasilitas pengelolaan sampah, fasilitas ruang publik, serta fasilitas jalan dan drainase

An Analysis Meanings of Hapo Ana (Thanksgiving for the Birth of a Child) Ritual Speech in Raerobo Communities of Sabu Raijua Regency: English

The purpose of this study was to describe the meaning contained in the Hapo Ana (thanksgiving for the birth a child) Ritual Speech in the community of Raerobo Village, Sabu Liae Subdistrict, Sabu Raijua District. The problem raised in this research is what are the meanings contained in the Hapo Ana (thanksgiving for the birth a child)  Ritual Speech in the Raerobo Village community, Sabu Liae Subdistrict, Sabu Raijua District. The theory used in this research is the theory of hermeneutics. The method used by the author in assessing the meaning in the ritual speech "Hapo Ana" (thanksgiving for the birth a child)  is a qualitative descriptive method. The results showed that the Hapo Ana (thanksgiving for the birth a child)  ritual ceremony consisted of several stages, namely (1) Atte ahhu (cutting the umbilical cord); (2) Ketoe nyabbu (hanging the placenta); (3) Pehune ngara  (naming) and (4) Hapo Ana “thaksgiving for the birth a child” (thanksgiving for the birth a child). &nbsp

Spatial and Functional Distribution of MYBPC3 Pathogenic Variants and Clinical Outcomes in Patients with Hypertrophic Cardiomyopathy

Background - Pathogenic variants in MYBPC3, encoding cardiac MyBP-C, are the most common cause of familial hypertrophic cardiomyopathy. A large number of unique MYBPC3 variants and relatively small genotyped HCM cohorts have precluded detailed genotype-phenotype correlations. Methods - Patients with HCM and MYBPC3 variants were identified from the Sarcomeric Human Cardiomyopathy Registry (SHaRe). Variant types and locations were analyzed, morphologic severity was assessed, and time-event analysis was performed (composite clinical outcome of sudden death, class III/IV heart failure, LVAD/transplant, atrial fibrillation). For selected missense variants falling in enriched domains, myofilament localization and degradation rates were measured in vitro. Results - Among 4,756 genotyped HCM patients in SHaRe, 1,316 patients were identified with adjudicated pathogenic truncating (N=234 unique variants, 1047 patients) or non-truncating (N=22 unique variants, 191 patients) variants in MYBPC3. Truncating variants were evenly dispersed throughout the gene, and hypertrophy severity and outcomes were not associated with variant location (grouped by 5' - 3' quartiles or by founder variant subgroup). Non-truncating pathogenic variants clustered in the C3, C6, and C10 domains (18 of 22, 82%, p<0.001 vs. gnomAD common variants) and were associated with similar hypertrophy severity and adverse event rates as observed with truncating variants. MyBP-C with variants in the C3, C6, and C10 domains was expressed in rat ventricular myocytes. C10 mutant MyBP-C failed to incorporate into myofilaments and degradation rates were accelerated by ~90%, while C3 and C6 mutant MyBP-C incorporated normally with degradation rate similar to wild-type. Conclusions - Truncating variants account for 91% of MYBPC3 pathogenic variants and cause similar clinical severity and outcomes regardless of location, consistent with locus-independent loss-of-function. Non-truncating MYBPC3 pathogenic variants are regionally clustered, and a subset also cause loss-of-function through failure of myofilament incorporation and rapid degradation. Cardiac morphology and clinical outcomes are similar in patients with truncating vs. non-truncating variants

Interação de um aluno com Paralisia Cerebral com colegas de classe durante atividades ludicas

Objetivou-se verificar a qualidade das relações interpessoais que se estabelecem entre uma criança com paralisia cerebral (PC) e seus colegas de classe durante atividades lúdicas em grupo realizadas em sala de aula e durante o recreio. Participaram deste estudo uma criança com diagnóstico clínico de PC quadriplégica, o professor e seus colegas de classe. A coleta de dados deu-se por meio de um roteiro de entrevista aplicado ao professor e por filmagens da criança com PC em atividades lúdicas com seus colegas. A análise de dados da entrevista foi feita de forma qualitativa e a análise da filmagem por meio de um sistema de categorias. Os resultados evidenciam que a professora tem a percepção de que a criança com PC tem interesse e participa de atividades lúdicas e interage satisfatoriamente não somente com parceiros habituais, mas com todos os colegas de classe. Por outro lado, a análise das filmagens evidencia que de fato a interação ocorre positivamente, porém a criança com PC permanece a maior parte do tempo mais como observadora das atividades do que como participante ativa, possivelmente em função da lacuna entre as possibilidades dessa criança e as demandas das atividades desenvolvidas no contexto escolar.The study aimed to examine the quality of interpersonal relationships developed between a child with cerebral palsy (CP) and his classmates during group play activities carried out in the classroom and during recess. The participants of the study were a boy with clinical diagnosis of quadriplegic CP, his teacher and classmates. Data collection was carried out by means of a structured interview with the teacher and of video recording the child with CP interacting with his classmates in play activities. The interview data analysis was carried out using qualitative procedures and the video recording was analyzed using a category system. The results showed that the teacher perceived the child with CP to be interested; he participated in play activities and interacted successfully both with familiar partners and with all classmates in general. On the other hand, the analysis of the video recorded activities showed that even though interaction did in fact occur positively, the child with CP remained mostly an observer of the activities rather than an active participant, possibly due to the gap between the child's possibilities and the requirements imposed by the activities underway in the school setting

Aspectos clínicos y epidemiológicos de los síndromes de Apert y Crouzon en España

Epidemiología y Teratología: Resultados sobre los datos del ECEMCApert and Crouzon syndromes are the most frequent ones among those syndromes with craniosynostosis. We have used data from the Spanish Collaborative Study of Congenital Malformations (ECEMC), to analyze some clinical and epidemiological characteristics of both syndromes. A total of 19 cases of Apert and 21 cases of Crouzon syndromes were identified among 1,914,726 liveborn infants. Their birth prevalence has diminished along the time although the decrease is only statistically significant for Crouzon syndrome. Mean parental ages, and mean differences between the parental ages, are significantly higher in Apert cases than in controls, what is indicating a relationship between paternal age and mutations for this syndrome. The differences between the parental ages of Crouzon cases and controls are not statistically significant. All the cases with Apert syndrome of our series were the first occurrence in the family, while 38.10% of the Crouzon cases were familial. We also observed a significant increasing linear trend in the frequency of infants with Apert syndrome, with the increasing paternal age, which is more noticeable since the age of 35 years. However, this is not observed for Crouzon cases whether sporadic or familial. Regarding the clinical aspects, Apert syndrome is more frequently associated to other defects than Crouzon syndrome. With respect to the affectation of hands in the Apert cases, the most frequent defect was the so-called "mitten hand", wich was present in 50% of our cases. In 28.57% the first finger was separated from the rest that are fused. Interestingly, in one case only the fifth finger was separated for the other four that remain fused.N