Renal amyloidosis in a child with sickle cell anemia
Tóm tắt
The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood.
Tài liệu tham khảo
Ataga KI, Orringer EP (2000) Renal abnormalities in sickle cell disease. Am J Hematol 63:205–211
De Santis Feltran L, de Abreu Carvalhaes JT, Sesso R (2002) Renal complications of sickle cell disease. Pediatr Drugs 4(1):29–36
Akar H, Keven K, Nergizoglu G, Erturk S, Ates K, Erbay B, Akar N, Duman N, Karatan O (2000) Renal amyloidosis in a patient with homozygous sickle cell anemia and M694V/M694V mutation. Nephron 86:383–384
Balal M, Paydas S, Seyrek N, Karayaylali I (2004) Different glomerular pathogenesis in sickle cell anemia. Clin Nephrol 62:400–401
Win N, Brozovic M, Gabriel R (1993) Secondary amyloidosis accompanying multiple sickle cell crisis. Trop Doct 23:45–46
Livneh A, Langevitz P, Zemer D (1997) Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 40(10):1879–1885
Stuart J, Stone PCW, Akinola NO, Gallimore JR, Pepys MB (1994) Monitoring the acute phase response to vaso-occlusive crisis in sickle cell disease. J Clin Pathol 47:16–166
Cotran RS, Kumar V, Robbins SL (1994) Diseases of immunity. In: Schoen FJ (ed) Pathologic basis of disease, 5th edn. Saunders, Philadelphia, pp 235
Connor JR, Menzies SL (1995) Cellular management of iron in the brain. J Neurol Sci 134:33–44
Simsek B, Islek I, Simsek T, Kucukoduk S, Cengiz K (2000) Regression of nephrotic syndrome due to amyloidosis secondary to familial Mediterranean fever following colchicine treatment. Nephrol Dial Transplant 15(2):281–282
Livneh A, Shtrasburg S, Langevitz P (2000) Regression of nephrotic syndrome in amyloidosis of familial Mediterranean fever following colchicine treatment. Nephrol Dial Transplant 15:1713–1714
Sarkissian A, Papazian M, Sanamyan A, Leumann E (2000) Colchicine in the treatment of renal amyloidosis secondary to familial Mediterranean fever. Nephrol Dial Transplant 15:1098