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

Bird Communities and Feeding Guilds From Three Land Use Types in Kerian River Basin, Perak

The objectives of this study were to determine the current bird species present in the study areas and to analyze variation of feeding guilds in different habitat types. Point count and mist-netting methods were used for bird census in SF and OP, while only point count method was used in PF. Feeding guilds were determined for each species recorded. A total of 183 species representing 49 families were recorded at all study sites. Secondary forest scored 106 species, followed by PF with 70 species and OP with 58 species. The highest abundance of bird families observed in SF was Pycnonotidae (26.65%), Hemiprocnidae (19.77%) and Muscicapidae (7.10%); in OP, Pycnonotidae (21.59%), Muscicapidae (19.12%) and Sturnidae (17.95%); in PF, Ardeidae (29.09%), Sturnidae (10.15%) and Hirundinidae (7.86%). Alternative hypothesis established that the abundance of species is different across habitat types, F(2,105) = 99.83, p < 0.05. Eight feeding guilds were identified in paddy field and seven in both secondary forest and oil palm plantation. If forests were converted to OP feeding guild could possibly change with a decreased in insectivore, insectivore-frugivore, insectivore-nectarivore and frugivore species; and increased in carnivore, grainivore and omnivore species. If forests were converted to PF feeding guild could possibly change with a decreased in insectivore, insectivore-frugivore and frugivore species; and increased in carnivore, insectivorenectarivore, insectivore-grainivore, grainivore and omnivore species. Comparison between SF and OP with Mann-Whitney U-test showed significant differences in six feeding guilds, including insectivore, insectivore-frugivore, insectivore-nectarivore, frugivore, grainivore and omnivore. Comparison between SF and PF showed significant differences in five feeding guilds, comprising of carnivore, insectivore-grainivore, frugivore, grainivore and omnivore. Comparison between OP and PF showed all feeding guilds to be significantly different. Complex vegetation in forestoffers more niches, with higher plant and insect diversity to support more bird species. In contrast, PF, a temporary wetland, support predominantly various waterbirds and migratory species

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

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