الفهرس 
IntroductionOnly 14 pages are availabe for public view
 |  | from | 125 |  |  |
| | | from | 125 | | |
Abstract
Renal manifestations in patients of CHA may range from minimal functional changes to very severe manifestations which require more therapeutic measures. The renal complications in CHA patients may affect the management and prognosis of CHA. As a consequence of red blood sickling in SCA, sickle cell nephropathy occurs by its tubular and glomerular presentations. The tubular manifestations are in the form of inability to concentrate the urine, mild tubular acidosis, impaired tubular secretion and excretion function such as impaired potassium secretion, increased sodium and phosphorus reabsorption and greater secretion of creatinine and urates On the other hand glomerular complications rang from hematuria, proteinuria and to lesser extent nephrotic syndrome. Also sickle cell nephropathy may lead to ESRD. For prevention of sickle cell nephropathy, patients with SCA must be routinely investigated by urine analysis, serum electrolytes and uric acid. Early detection of patients at high risk of developing renal tubular and glomerular dysfunctions is very important to reduce the incidence of ESRD. This early detection can be done by regular follow up of serum levels of Cys-C, β2M and urinary excretion of β2M and NAG. As regarding some points of management of sickle cell nephropathy, hematuria is managed first by exclusion of Infections, malignancy, coagulopathy and other causes of hematuria then increasing the fluid intake, alkalinization of the urine and may need to blood transfusion or blood substitutes if it is necessary and other measures to stop bleeding. Proteinuria and to lesser extent nephrotic syndrome are not treated in this case by prednisone or cyclophosphamide. ACE inhibitors decrease proteinuria. Hemodialysis and renal transplantation can be performed with success in patients with ESRD resulting from SCA. The incidence of complications related to hemodialysis does not significantly differ in patients with sickle cell nephropathy from that observed in the general population. In β thalassemia patients, there is a high prevalence of renal tubular abnormalities. The underlying mechanism for renal dysfunctions seem to be multifactorial, attributed mainly to include long-standing anemia, chronic hypoxia, iron overload, deferoxamine toxicity and vascular thrombosis and renal infarction due to increased platelet aggregation and decreased serum level of protein S and antithrombin III. Impaired tubular secretion, excretion function and other tubular dysfunctions are associated with different types of thalassemia. Glomerular diseases may also develop; IgA nephropathy was reported in a patient with thalassemia major. Management of renal complications of thalassemia not differs than that with SCA. Renal complications may occur as adverse effect of treatment of CHA especially iron chelation therapy and not only as a complication of CHA itself. So patients on iron chelation therapy must be investigated regularly for early detection of any renal abnormalities.