[Hereditary fibrinogen Aα-chain amyloidosis caused by the E526V mutation: a case report and literature review].

Mutations in the fibrinogen Aα-chain genes are the most common cause of hereditary renal amyloidosis. The renal histologic appearance in the patient is characteristic and shows striking glomerular enlargement with almost complete obliteration of the normal glomerular architecture by extensive amyloid deposition. In contrast, the vessels and renal tubular interstitium of such patient contains almost no amyloid at all.

Acquired Fanconi syndrome with proximal tubular cytoplasmic fibrillary inclusions of λ light chain restriction.

Light chain proximal tubulopathy is a rarely reported entity associated with plasma cell dyscrasia that classically manifests as acquired Fanconi syndrome and is characterized by the presence of κ-restricted crystals in the proximal tubular cytoplasm. We herein present a case of multiple myeloma with Fanconi syndrome and acute kidney injury due to light chain proximal tubulopathy with light chain cast nephropathy. Prominent phagolysosomes and numerous irregularly shaped inclusions with a fibrillary matrix in the cytoplasm of the proximal tubules were identified on electron microscopy.

A clinicopathological analysis in a large cohort of Chinese patients with renal amyloid light-chain amyloidosis.

Background: The aim of the study is to investigate the association between clinical and pathological features in a large cohort of Chinese patients with renal immunoglobulin light-chain amyloidosis (AL).

Methods: A series of 186 patients with renal AL amyloidosis diagnosed between 1990 and 2011 were retrospectively reviewed. The extent of amyloid deposition in glomeruli, blood vessels and tubulointerstitium were evaluated semiquantitatively.

Diffuse thin glomerular basement membrane in association with Fabry disease in a Chinese female patient.

We report a 41-year-old Chinese female with Fabry disease and diffuse thinning of the glomerular basement membrane (GBM). The patient presented with peripheral edema, mild proteinuria, microscopic hematuria, normal renal function, hypertension and tinnitus. Family screening showed that her daughter had microscopic hematuria, tinnitus and neuropathic pain.

Ultrastructural features and expression of cytoskeleton proteins of podocyte from patients with minimal change disease and focal segmental glomerulosclerosis.

Focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) have been suggested for the category of podocytopathies. An ultrastructural observation and immunogold labeling for cytoskeleton proteins of podocytes on 11 cases each of FSGS and MCD were performed. Compared to MCD, more severe ultrastructural alterations of podocyte were identified in FSGS, which were characterized by higher frequency of mat-like condensation of microfilaments in the foot process and the detachment of the foot process from glomerular basement membrane.

[Clinicopathologic study of different variants of focal segmental glomerulosclerosis].

Objective: To study the clinicopathologic features of different variants of primary focal segmental glomerulosclerosis (FSGS).

Methods: One hundred and two cases of FSGS were retrieved from the archival files of Peking University First Hospital during the past 6-year period. The pathologic findings were reviewed and the degrees of active and chronic changes were assessed by morphometric analysis.

Antineutrophil cytoplasmic autoantibodies in patients with systemic lupus erythematosus recognize a novel 69 kDa target antigen of neutrophil granules.

Objective: Antineutrophil cytoplasmic autoantibodies (ANCA) were found in patients with systemic lupus erythematosus (SLE). Cathepsin G and lactoferrin were the major target antigens. However, some ANCA-positive sera did not recognize either of them.

Autoantibodies against glomerular mesangial cells and their target antigens in lupus nephritis.

Unlabelled: Mesangial proliferation and deposition of immunoglobulins and complement components within glomerular mesangium was one of the important pathological features of lupus nephritis. Autoantibodies against human mesangial cells could be detected in the sera of patients with IgA nephropathy (IgAN) and Henoch-Schöenlein nephritis. We speculated that autoantibodies against human glomerular mesangial cells might play a role in the development of lupus nephritis.

[Anti-endothelial cell antibodies(AECA) are associated with disease activity of propylthiouracil induced antineutrophil cytoplasmic antibody (ANCA) positive vasculitis].

Objective: To investigate the prevalence of anti-endothelial cell antibodies(AECA) and its possible role in the pathogenesis of propylthiouracil (PTU) induced ANCA positive vasculitis.

Methods: Sera from 11 patients with PTU induced ANCA positive vasculitis and 10 patients with PTU induced ANCA but without clinical vasculitis were studied. Soluble proteins from in vitro cultured human umbilical vein endothelial cells were used as antigens and immunoblotting technique was performed to identify the specific target antigens.

The clinical and pathological characteristics of Chinese elderly patients with anti-neutrophil cytoplasmic autoantibodies associated small vessel vasculitis.

Background: Anti-neutrophil cytoplasmic autoantibodies (ANCA) are serological markers of ANCA-associated small vessel vasculitis (AASV) which is one of common autoimmune diseases in Caucasian elderly population.

Objective: To analyze the clinical and pathological characteristics of Chinese elderly patients with AASV.

Methods: One-hundred forty one Chinese patients with AASV over 65 years old, diagnosed between 1997 and 2001 in the Institute of Nephrology of Peking University First Hospital, were retrospectively studied and their clinical and pathological data were analyzed.

[The clinic significance of urinary podocytes in patients with focal segmental glomerulosclerosis].

Objective: To address the significance of urinary podocytes in the diagnosis of human focal segmental glomerulosclerosis(FSGS).

Methods: Twelve patients with FSGS and 20 patients with minimal change disease (MCD) were diagnosed by routine renal biopsy, and 8 healthy persons as controls. Morning urinary sediments was collected and centrifuged onto glass slides.

[Clinical and pathological features of anti-neutrophil cytoplasm antibody associated systemic vasculitis in children].

Objective: Anti-neutrophil cytoplasmic antibodies (ANCA) are serological diagnostic markers for certain types small vessel vasculitis including Wegener's granulomatosis and microscopic polyangiitis, which are also termed ANCA associated systemic vasculitis (AASV). The majority of patients with primary AASV reported are adults and predominantly elderly. Data on pediatric patients with primary AASV in China are lacking.

[The relationship between subclassification of anti-myeloperoxidase IgG by enzyme-linked immuno-sorbent assay analysis and vasculitis activity].

Objective: Antineutrophil cytoplasmic antibody (ANCA) is an important serological diagnostic tool for certain vasculitides, such as Wegener's granulomatosis and microscopic polyangiitis. Several studies suggested that ANCA titers correlated with disease activity. However, in some patients, ANCA still remained positive when patients were in clinical remission.