Beta-thalassemia: renal complications and mechanisms: a narrative review.
Hematology
; 24(1): 426-438, 2019 Dec.
Article
in En
| MEDLINE
| ID: mdl-30947625
ABSTRACT
OBJECTIVES:
Beta-thalassemias are a group of recessively autosomal inherited disorders of hemoglobin synthesis, which, due to mutations of the beta-globin gene, lead to various degrees of defective beta-chain production, an imbalance in alpha/beta-globin chain synthesis, ineffective erythropoiesis, and anemia. Improved survival in thalassemic patients has led to the emergence of previously unrecognized complications, such as renal disease.METHODS:
A comprehensive literature review through PubMed was undertaken to summarize the published evidence on the epidemiology and pathophysiology of renal disease in thalassemia. Literature sources published in English since 1990 were searched, using the terms beta-thalassemia, renal disease.RESULTS:
Renal disease is considered to be the 4th cause of morbidity among patients with transfusion dependent thalassemia. Chronic anemia, hypoxia and iron overload are the main mechanisms implicated in development of renal injury, whereas several studies also suggested a contributive role of iron chelators. DISCUSSION ANDCONCLUSION:
Kidney disease may develop through progressive renal tubular and glomerular damage; thus, its early recognition is important in order to prevent and/or reverse deterioration. This review will provide an insight on the involved mechanisms implicated in kidney disease in thalassemic patients and will discuss the updates on diagnosis and prevention of renal complications in thalassemia.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Beta-Thalassemia
/
Iron Overload
/
Kidney Diseases
/
Hypoxia
Type of study:
Etiology_studies
/
Systematic_reviews
Limits:
Female
/
Humans
/
Male
Language:
En
Journal:
Hematology
Journal subject:
HEMATOLOGIA
Year:
2019
Document type:
Article
Affiliation country:
Greece