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ArtikelAnemia Sel Sabit dan Hidroksi Urea sebagai Salah Satu Terapi Pilihan  
Oleh: Ridwan, Rahmawati
Jenis: Article from Journal - ilmiah nasional - tidak terakreditasi DIKTI
Dalam koleksi: Widya: Majalah Ilmiah vol. 23 no. 250 (Jul. 2006), page 52-57.
Topik: Anemia; Anemia Sel Sabit; Hidroksi Urea; Terapi Pilihan
Ketersediaan
  • Perpustakaan Pusat (Semanggi)
    • Nomor Panggil: MM47.24
    • Non-tandon: 1 (dapat dipinjam: 0)
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  • Perpustakaan PKPM
    • Nomor Panggil: W36
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Isi artikelSickle cell disease (SCD) and its variants are genetic disorders of mutant hemoglobins (Hb). The most common form is found in North America known as HbS disease. HbS arises from a mutation that substiitutes Thymine for Adenine that causes coding of Valine instead of Glutamine in position 6 of the beta chain. The resulting HbS exhibits changes in solubility and molecular stability. When homozygous HbS are exposed tro deoxy conditions, the cycling process begins. The polymer formation follows, and the RBC assumes a characteristic sickle-shape, membrane damage occurs, and the cell becomes permanently sickle-shaped. The physiological changes leads to hemolytic anemia, painful vasooccclusive crisis, and multiple organ damage with micro infarcts incluading ehart, skeleton, spleen and central nervous system. Treatment with hydroxyl-urea increases the production of HbF and lowers the frequency as well as severity of vaso-occlusive crisis. The production of cocoa in Indonesia and also caused a decrease in production surplus and increased consumer surplus
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