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"Thalassemia b mayor adalah penyakit yang disebabkan oleh kelainan sintesis rantai polipeptida b yang diturunkan secara otosom resesif. Pengobatan thalassemia b mayor pada umumnya berupa pemberian transfusi berulang, yang mengakibatkan penumpukan besi dan berakhir dengan hemokromatosis. Penumpukan besi dapat terjadi pada organ tubuh antara lain ginjal. Tujuan penelitian ini untuk mengetahui adanya gangguan fungsi ginjal pada penderita thalassemia b mayor berumur 15-28 tahun yang telah mendapatkan 6 unit packed red cells. Pada penelitian ini telah diperiksa kadar besi serum (SI) dan daya ikat besi total (TIBC) serta kadar mikroalbumin dan b2-mikroglobulin (b2-m) dalam urin. Hasil yang didapat 94,7% penderita menunjukkan peningkatan saturasi transferin dan 40% diantaranya disertai hemokromatosis; 73,4% disertai mikroalbuminuria, 1,3% dengan albuminuria dan 21,3% dengan peningkatan b2-m urin. Jumlah kasus dengan kelainan ginjal dijumpai pada 78,6%. (Med J Indones 2003; 12: 215-223)

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b-thalassemia major is a disease caused by b polypeptide chain synthesis disorder which is inherited in an autosomal recessive manner from both parents and which is marked by little or no b-globin chain synthesis. Treatment for b-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 b-thalassemia major patients. The subjects of this study were b-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 b2-microglobulin (b2-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)"

"Pada dua tahun pertama kehidupan penderita talasemia mayor, umumnya menderita anemia dan membutuhkan tranfusi darah. Penelitian ini bertujuan membuat model prediksi kebutuhan darah bagi penderita talasemia mayor. Penelitian observasional dengan desain studi pendekatan potong lintang ini dilakukan pada sampel 79 penderita talasemia mayor yang melakukan transfusi rutin minimal satu bulan satu kali di Rumah Sakit Umum Banyumas, selama tahun 2012. Analisis regresi linier ganda digunakan untuk membuat model prediksi. Hasil penelitian menunjukkan bahwa 80,7% kebutuhan darah penderia talasemia mayor dijelaskan oleh variabel usia, berat badan, dan kadar hemoglobin sedangkan 19,3% dijelaskan oleh sebab-sebab yang lain. Rumus prediksi menyatakan setiap kenaikan usia 1 tahun maka kebutuhan darah akan bertambah sebanyak 0,816 mililiter dan setiap kenaikan 1 kilogram berat badan maka kebutuhan darah akan bertambah 13,4 mililiter serta apabila kadar hemoglobin mengalami penurunan 1 g/dL maka kebutuhan darah akan bertambah sebesar 81 mililiter.

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Patients with thalassemia major usually present within the first two years of life with severe anemia, need red blood cell transfusion. The objective of this study was to create a prediction model of blood transfusion need for patients with thalassemia mayor. This type of research was observational with cross sectional design. Samples are 79 patients with thalassemia major who perform routine transfusion at least once in a month at Banyumas hospital in 2012. Multiple linier regression analysis was used to create the model. The results showed that 80.7% blood requirements can be explained by variables of weight, haemoglobin level and age, while 19.3% is explained by other causes. Prediction formula states every increment of one year in age, the need for blood will increase by 0.816 millilitres and every increment of one kilogram of body weight, the need for blood will increase 13.4 millilitres, Model Prediksi Kebutuhan Darah untuk Penderita Talasemia Mayor Blood Need Prediction Model for Mayor Thalassemia Patients Dwi Sarwani Sri Rejeki* Putri Pradani* Nunung Nurhayati** Supriyanto** and when the haemoglobin level decreased 1 gr/dL the need for blood will increase by 81 millilitres."