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Simon Kusnandar
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Medicine & Pharmacology

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Diabetes mellitus in β-thalassemia major patients Wirawan, Riadi; Setiawan, Santy; Kusnandar, Simon; Munthe, Bulan G.
Medical Journal of Indonesia Vol 12, No 2 (2003): April-June
Publisher : Faculty of Medicine Universitas Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (204.407 KB) | DOI: 10.13181/mji.v12i2.94

Abstract

β-thalassemia major is a disease caused by β polypeptide chain synthesis disorder which is inherited as an autosomal recessive from both parents which is marked by little or no β globin chain synthesis. Medication for β thalassemia major patients is by repeated blood transfusions, which causes hemochromatosis. Hemochromatosis can occur in various organs including the pancreas. The aim of the study was to assess the alteration of plasma glucose concentration and the hemochromatosis prevalence. Fasting plasma glucose concentration and serum ferritin examination were measured in 115 β thalassemia major patients with ages between 10-23 years who were out-patients in the Thalassemia Centre, Department of Child Health, Medical School, University of Indonesia / Dr. Cipto Mangunkusumo General Hospital, Jakarta. The plasma glucose concentration examination was conducted by the GDH enzymatic method, with American Diabetes Association (ADA) criteria in the evaluation, while the serum ferritin examination was conducted with the microparticle enzyme immuno assay (MEIA) method. All patients had hemochromatosis, 14.8% of the patients had impaired fasting glucose level and 2.6% of the patients showed indications of diabetes mellitus. β thalassemia major patients who receive frequent transfusions will develop hemochromatosis that will in turn impair the pancreatic function. (Med J Indones 2003; 12: 87-93) Keywords : β thalassemia major, hemochromatosis, diabetes mellitus
Renal impairment in β thalassemia major patients receiving repeated blood transfusion Wirawan, Riadi; Kusnandar, Simon; Suherli, Abas; Gatot, Djajadiman
Medical Journal of Indonesia Vol 12, No 4 (2003): October-December
Publisher : Faculty of Medicine Universitas Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (215.146 KB) | DOI: 10.13181/mji.v12i4.114

Abstract

β-thalassemia major is a disease caused by β polypeptide chain synthesis disorder which is inherited in an autosomal recessive manner from both parents and which is marked by little or no β-globin chain synthesis. Treatment for β-thalassemia major patients is by giving repeated blood transfusions, which causes iron accumulation, leading to hemochromatosis. Iron accumulation can occur in various body organ, including the kidneys. The aim of this study was to investigate the existence of renal impairment in β-thalassemia major patients. The subjects of this study were β-thalassemia major patients aged 15 - 28 years old who had received 6 units of packed red cells or more within 6 months. In this study, urine and serum samples of the subjects were taken and examined. Assay of serum iron was performed with Hitachi 737. Results were that 94.7% patients showed an increase in transferrin saturation and 40% of them had hemochromatosis; 73.4% had microalbuminuria; 1.3% had albuminuria and 21.3% had increased urinary β2- microglobulin (β2-m). A total of 78.6% of patients showed renal impairment. Conclusion of this study suggested that glomerular dysfunction happens in an earlier stage of the disease process. The high incidence of microalbuminuria is also attributed to defective ability of the proximal tubular cells to reabsorb protein besides dysfunction of the glomeruli. (Med J Indones 2003; 12: 215-223)Keywords: β-thalasemia major, repeated blood transfusion, renal impairment
Co-Authors Abas Suherli Bulan G. Munthe Djajadiman Gatot Riadi Wirawan Santy Setiawan