Mechanisms of Renal Disease in β-Thalassemia

Abstract

Although advances in the care of patients with b-thalassemia translate into better patient survival, this success has allowed previously unrecognized complications to emerge, including several renal abnormalities. Clinical studies continue to show that mild tubular dysfunction and abnormalities in GFR are common in patients with b-thalassemia. Chronic anemia and iron overload are believed to lie behind these abnormalities. Nonprogressive increases in levels of serum creatinine have also been observed after exposure to some iron chelators. Longitudinal studies are needed to understand the true burden of renal dysfunction in patients with b-thalassemia.

Reversal of hemochromatosis by apotransferrin in non-transfused and transfused Hbbth3/+ (heterozygous b1/b2 globin gene deletion) mice

“…However, recent reviews as well as clinical and basic research suggest that renal disease may be a consequence of longer survival of patients. [40][41][42] While several factors, including anemia-induced hypoxia, iron overload and iron chelation, may be responsible, 41 it is logical that iron overload was the cause of the pathological changes in our model since chronic anemia is unlikely in intermediate Hbb th3/+ mice and no small molecule chelators were administered. We speculate that one cause of the reduction in tissue iron may be the ability of apoTf to decrease NTBI in the plasma and tissue parenchyma.…”

Role of serum cystatin-C and beta-2 microglobulin as early markers of renal dysfunction in children with beta thalassemia major

“…1 With advances in treatment and chelating agents of β-TM patients, new unrecognized complications have emerged, such as renal complications. 2 Many factors contribute to the functional abnormalities found in β-TM patients such as decreased red cell life span, rapid iron turnover, and tissue deposition of excess iron and also, specific iron chelators can affect kidneys. 3 β-TM patients usually suffer from several complications like cardiopulmonary, reticuloendothelial and other major systems dysfunctions.…”

Perbandingan Kadar N-Acetyl-ß-D-Glucosaminidase Urin pada Thalassemia ß Mayor Anak yang Mendapat Deferipron dan Deferasiroks

“…Apabila besi memasuki sel tubulus ginjal masih berikatan dengan transferin, pelepasan besi terjadi dalam lisosom dan memasuki sitoplasma dalam bentuk besi bebas yang reaktif, dapat memproduksi spesies oksigen reaktif dan jejas sel. 3 Anemia kronik berkaitan dengan stres oksidatif yang mengakibatkan peroksidasi lipid dan abnormalitas fungsi sel tubulus. 15 Penelitian yang dilakukan oleh Sumboonnanonda dkk 6 menunjukkan adanya korelasi antara abnormalitas tubulus dengan derajat anemia.…”

“…2 Gangguan fungsi ginjal pasien TßM dapat terjadi pada tingkat glomerulus ataupun tubulus. 3 Kelainan tubulus ginjal merupakan kelainan patologi yang lebih banyak dijumpai pada biopsi ginjal thalassemia. 4 Kelebihan besi dan anemia kronik merupakan faktor utama penyebab abnormalitas tubulus ginjal.…”

“…4 Kelebihan besi dan anemia kronik merupakan faktor utama penyebab abnormalitas tubulus ginjal. 1,3,5,6 Pada kasus TßM, beberapa penelitian tentang disfungsi ginjal memperlihatkan bahwa pemeriksaan biokimia rutin tidak dapat mendeteksi dini kerusakan tubulus ginjal. 7,8 Penelitian lain membuktikan bahwa N-acetyl-β-D-glucosaminidase (NAG) merupakan pemeriksaan yang paling dapat diandalkan untuk mendeteksi dini kerusakan tubulus ginjal pada TßM dibandingkan dengan pemeriksaan yang lain.…”