Autologous immune complex nephritis associated with sickle cell trait: diagnosis of the haemoglobinopathy after renal structural and immunological studies.
نویسندگان
چکیده
A renal tubular epithelial antigen (RTE)--anti-RTE autologous immune complex nephritis associated with sickle cell anaemia (SS) has been reported, but immune complex nephritis has never been described in patients with sickle cell trait (SA). During investigation of a child with "asymptomatic proteinuria" cryoprecipitable complexes of RTE-anti-RTE were detected in the serum and granular deposits of RTE, immunoglobulins, and complement localised on the glomerular basement membranes. Morphological and ultrastructural studies showed increased mesangial matrix, sickled red blood cells in the glomeruli and vessels, and tubular and interstitial abnormalities. These findings prompted haemoglobin electrophoretic studies, which showed previously undiagnosed haemoglobin SA in this patient and her family. These observations suggest that nephritis mediated by similar immunopathogenic mechanisms may be associated with SS and SA haemoglobinopathy. Under some conditions patients with sickle cell trait may experience haemodynamic and oxygenation abnormalities, which may be aetiological factors in the immune complex nephritis associated with SS disease.
منابع مشابه
Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?
Background: Until now, trimodal distribution of HbS has been seen by six different studies in the world when associated with alpha-thalassemia with confirmation by corresponding alpha-genotyping studies. The RBC indices reduce as alpha-globin genes reduce in sickle cell trait (SCT) patients, which decreases the extent of intra-vascular sickling and thus betters the clinical cou...
متن کاملPre-marriage Sickle Cell Screening Program in South Region of Iran, A Pilot Study on 50 Cases of Sickle Trait
Background: Studies have demonstrated that sickle cell trait can be found in an asymptomatic healthy carrier with normal complete blood count (CBC) and red blood cell (RBC) indices. According to Iranian Ministry of Health bulletin instructions, prenuptial Thalassemia Screening Program (TSP) primarily depends on RBC indices which are measured through a routine CBC. Only when these levels are bel...
متن کاملEvaluation of Therapeutic Effects of Autologous Bone Marrow Mesenchymal Stem Cells to Prevent the Progression of Chronic Nephropathy in Renal Transplant
Background Chronic allograft nephropathy(CAN) is one of the most common causes of chronic and end stage renal disease. It is defined with Mainly tubular atrophy and interstitial fibrosis and no evidence of any other etiology, or functional disorder that caused at least three months after transplantation . Control of risk factors (HTN,DM,HLP, …) and limiting usage of calcineurin inhibitors...
متن کاملGlucose-6-Phosphate Dehydrogenase Deficiency Associated with Thalassemia and Sickle Cell Anemia in an Iranian Family
Sickle cell-thalassemia associated with glucose- 6-phosphate dehydrogenese deficiency is reported in an Iranian family. The father had sickle trait and G.6.P.D. deficiency, the mother minor thalassemia, one of the sons siclc:le ceH-thailasemiia., the oth-er sickle cell trait; the daughter had sickle cell-thalassemia and was carrier of G.6.P.D. deficiellcy.
متن کاملCoexistence of lupus nephritis and sickle cell trait, an electron microscopic assessment of renal glomerular damage: Case report of a rare association
Because of similarities of the musculoskeletal, central nervous system, and renal manifestations in both diseases, diagnosing systemic lupus erythematosus (SLE) in sickle cell disease (SCD) patients can be difficult to establish. Although Sickle Cell Trait (SCT) is still considered a benign form of SCD, its impact on kidney injury and other renal manifestations is well recognized in the literat...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- British medical journal
دوره 1 6006 شماره
صفحات -
تاریخ انتشار 1976