Emerging treatment approaches for the systemic amyloidoses.

نویسنده

  • Laura M Dember
چکیده

A 57-year-old man was diagnosed with AL amyloidosis after presenting with nephrotic syndrome. He had been well until 6 months earlier, when he noticed intermittent ankle swelling. He sought medical attention approximately 2 months later because of increased swelling of his legs and progressive fatigue. At that time, his urinary protein excretion was 7.4 g/day. Serum creatinine was 0.9 mg/dL and serum albumin 3.1 g/dL. A renal biopsy disclosed amorphous material in the mesangium that was birefringent under polarized light when stained with Congo red dye. Immunofluorescence was positive for lambda light chain in the glomerular capillary walls, mesangium, and tubulointerstitium. Electron microscopy revealed randomly dispersed fibrils with a diameter of 10 nm throughout the mesangium and interstitium. A monoclonal IgG lambda protein and a monoclonal free lambda light chain were evident by immunofixation electrophoresis (IFE) of the serum and urine, respectively. Bone marrow biopsy had 5% plasma

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

New insights into systemic amyloidosis: the importance of diagnosis of specific type.

PURPOSE OF REVIEW This review aims to summarize recent developments in the area of systemic amyloidoses with emphasis on pathologic diagnosis. RECENT FINDINGS In recent years, management of amyloidosis has shifted from a purely supportive approach to quite diverse, radical and aggressive treatments. The central issue is the understanding that treatment of systemic amyloidoses depends on the m...

متن کامل

Mouse Models to Study Systemic Amyloidoses: Is Prion-Like Transmission a Common Pathogenic Mechanism?

The amyloidoses are a group of protein-misfolding disorders characterized by the accumulation of amyloid fibrils formed from a variety of proteins. Currently, twentyeight different kinds of human and animal proteins, in intact or fragmented forms, have been found to be associated with pathological disorders such as Alzheimer’s disease, type II diabetes, prion diseases, dialysis-related amyloido...

متن کامل

Curcumin: A multi-target disease-modifying agent for late-stage transthyretin amyloidosis

Transthyretin amyloidoses encompass a variety of acquired and hereditary diseases triggered by systemic extracellular accumulation of toxic transthyretin aggregates and fibrils, particularly in the peripheral nervous system. Since transthyretin amyloidoses are typically complex progressive disorders, therapeutic approaches aiming multiple molecular targets simultaneously, might improve therapy ...

متن کامل

Systemic amyloidosis: are we moving ahead?

Systemic amyloidoses are a wide group of diseases with different courses, treatments and prognoses. Unequivocal typing of amyloid deposits is important for correct diagnosis and appropriate treatment. At present, the most effective therapeutic approach is based on eliminating the supply of amyloidogenic precursor. New effective therapies will stem from our improved knowledge of the molecular me...

متن کامل

Cutaneous Septic Embolism Presenting as Erythematous Plaques

392 Ann Dermatol Received June 12, 2012, Revised November 8, 2012, Accepted for publication November 22, 2012 Corresponding author: Javier Galve, Department of Dermatology, Hospital Clínic, C/ Villarroel 170, 08036 Barcelona, Spain. Tel: 34-93-2275400 (Ext 2358), Fax: 34-93-2275438, E-mail: [email protected] This is an Open Access article distributed under the terms of the Creative Commons...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Kidney international

دوره 68 3  شماره 

صفحات  -

تاریخ انتشار 2005