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Iswari Setianingsih
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Applicability of a clinical scoring criteria for disease severity of ß-thalassemia/hemoglobin E in Indonesia Wahidiyat, Pustika A.; Sastroasmoro, Sudigdo; Fucharoen, Suthat; Setianingsih, Iswari; Putriasih, Siti A.
Medical Journal of Indonesia Vol 27, No 1 (2018): March
Publisher : Faculty of Medicine Universitas Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (274.528 KB) | DOI: 10.13181/mji.v27i1.1779

Abstract

Background: β-thalassemia/HbE presents with a variety of clinical symptoms, from asymptomatic to severe, requiring routine transfusion. However, there is currently no agreed classification system to stratify patients based on clinical severity of β-thalassemia/HbE in the Indonesian population. Thailand has already established a classification system, and this study aimed to identify the applicability of the Thailand clinical scoring criteria to determine the severity of β-thalassemia/HbE in the Indonesian population.Methods: This descriptive study was conducted by evaluating patients with β-thalassemia/HbE, who were classified into mild, moderate, or severe groups based on the Thailand clinical scoring criteria.Results: A total of 293 subjects with β-thalassemia/HbE were included. Based on this clinical scoring criteria, it was found that only 21.5% of patients were classified as mild, and the remaining 35.5% and 44% were classified as moderate and severe respectively. Approximately 68.2% of the subjects in the severe group received transfusion at <4 years old, while only 10% of those in the mild group were transfused at the same age. In the mild group, only 10% of the subjects underwent routine transfusion, compared to 98.4% of the subjects in the severe group. In addition, only 27% of the subjects in the mild group showed stunted growth, while that in the moderate and severe groups were 54.5% and 86.8%, respectively.Conclusion: Thailand clinical scoring criteria is able to determine the severity of Indonesia thalassemia patient which needs further management, i.e. transfusion and observation of stunted growth. This scoring system will help provide the provision of the most ideal management for the groups of patients based on their requirements.
Hubungan antara Tipe Mutasi Gen Globin dan Manifestasi Klinis Penderita Talasemia Tamam, Moedrik; Hadisaputro, Suharyo; Sutaryo, Sutaryo; Setianingsih, Iswari; Astuti, Rini; Soemantri, Agustinus
Jurnal Kedokteran Brawijaya Vol 26, No 1 (2010)
Publisher : Fakultas Kedokteran Universitas Brawijaya

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (543.469 KB) | DOI: 10.21776/ub.jkb.2010.026.01.9

Abstract

ABSTRAKThalassemia β di populasi umum  mempunyai manifestasi klinis  yang sangat bervariasi mulai dari thalassemia ringan sampai berat. Penentuan  jenis  mutasi adalah penting  karena pengetahuan tentang tipe mutasi yang mendasari thalassemia-β diperlukan dalam pengelolaan pasien. Penelitian ini bertujuan untuk menilai hubungan antara  tipe mutasi dengan manifestasi klinis penderita thalassemia. Penelitian ini merupakan penelitian observasional kohort prospective dilaksanakan di Unit Transfusi Darah  (UTD PMI Kota Semarang mulai bulan Januari 2006-Desember 2009. Sampel menggunakan 38 pasien thalassemia  yang dipilih secara purposive sampling dari penderita thalassemia β yang menerima transfusi. Variabel penelitian adalah jenis mutasi gen globin , manifestasi klinis, status gizi, dan kadar Hb sebelum dan sesudah transfusi. Pengukuran kadar Hb dilakukan sebelum transfusi ke-1, setelah transfusi ke-1 dan sebelum transfusi ke-22. Analisis statistik menggunakan uji  dan uji Fisher-exact. Rerata umur subyek penelitian adalah 10,1 ± 3,26 tahun, laki-laki sebesar  16 orang (42,1%) dan perempuan 22 orang (57,9%). Jenis mutasi adalah HbE/ivsnt1-nt5 (55,3%), ivs1-nt5 dan HbE CD35 (masing-masing 13,2%), HbE/ivs1-nt1 (7,9%), dan ivs5-nt1/ivs1, ivs1-nt1/ivs1-nt1, HbE/CD41-42 dan exon 1-2 normal (masing-masing 2,6%). Manifestasi klinis derajat sedang 65,8% dan berat 34,2%. Hasil uji statistik menunjukkan hubungan tidak bermakna antara jenis mutasi dengan manifestasi klinis thalassemia (p=0,5). Jenis tipe mutasi gen globin  tidak berhubungan dengan derajat manifestasi klinis thalassemia.Kata kunci : Manifestasi klinis, talasemia , tipe mutasi
Hubungan antara Stres Oksidatif dengan Kadar Hemoglobin pada Penderita Thalassemia/Hbe Tamam, Moedrik; Hadisaputro, Suharyo; Sutaryo, Sutaryo; Setianingsih, Iswari; Djokomoeljanto, Djokomoeljanto; Soemantri, Ag
Jurnal Kedokteran Brawijaya Vol 27, No 1 (2012)
Publisher : Fakultas Kedokteran Universitas Brawijaya

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (537.57 KB) | DOI: 10.21776/ub.jkb.2012.027.01.7

Abstract

Pemberian  transfusi  darah  kronik  pada  penderita  thalassemia  dapat   menyebabkan  kelebihan  kadar  besi.  Kelebihan  kadar besi  bebas  dalam  tubuh  akan  memacu  timbulnya  oksidan  berupa  reactive  oxygen  species   (ROS)  yang  diukur  dalam  bentuk malondialdehide  (MDA).  Peningkatan  produksi  ROS  dapat  menyebabkan  kerusakan  membran  sel  yang  mengandung senyawa lipid    termasuk eritrosit. T ujuan penelitian ini  adalah untuk menilai hubungan antara kadar MDA serum dengan kadar Hb penderita thalassemia. Metode penelitian ini  adalah penelitian observasional kohort prospective dilaksanakan di  UTD  PMI  Kota  Semarang.  Selama  bulan  Januari  2006  sampai  dengan  Desember  2009  dijumpai  32  penderita  thalassemia/HbE.  Variabel  penelitian kadar Hb dan MDA   serum.  Pengukuran dilakukan sebelum  transfusi  ke-1, setelah transfusi  ke-1 dan sebelum transfusi  ke-2. Analisis statistik menggunakan uji Mann-Whitney,  uji Wilcoxon  dan uji korelasi Spearman. Rerata  umur  subjek  penelitian  adalah  9,5±  3,13  tahun,  laki-laki  adalah  25  orang  (46,9%)  dan  perempuan  17  orang  (53,1%). Status  gizi  43,8  %  termasuk  gizi  kurang.  Kadar  MDA   kelompok   Hb  sebelum  transfusi  ke-2  8  g/dL  2,89±0,451  dan  kelompok Hb  >8  g/dL  2,19±0,792  (p=0,01).  Ada  korelasi  negatif  antara  Hb  sebelum  transfusi  ke-2  dengan  MDA  (r=min  0,52;  p=0,002). Dapat  disimpulkan  ada  korelasi  negatif  antara  kadar  MDA  serum  dengan  kadar  Hb  pada  penderita  thalassemia  /HbE. Kata  Kunci:  Hemoglobin,  MDA,  stres  oksidatif ,  thalassemia  /HbE
Co-Authors Ag Soemantri Agustinus Soemantri Djokomoeljanto Djokomoeljanto Fucharoen, Suthat Moedrik Tamam Pustika A. Wahidiyat Putriasih, Siti A. Rini Astuti Sudigdo Sastroasmoro Suharyo Hadisaputro Sutaryo Sutaryo