中国医大儿科学5c.pdf

Nephrotic Syndrome Nephrotic Syndrome Nephrotic syndrome(NS) results from increased permeability of GBM to plasma protein. It is characterized by excessive proteinuria, hypo- proteinemia, hypercholesterolemia and edema. 1.Types of nephritic syndrome: (1) Idiopathic nephritic syndrome: Etiology of the disease is unknown, accounting for approxi- mately 90% of nephrosis in childhood. (2) Secondary nephrosis: NS resulted from systemic disease such as anaphylactoid purpura, systemic lupus erythematosis (SLE), and so on. 2.Etiology The cause of the idiopathic NS remains unknown. Recent 10 years increasing evidence has suggested that the syndrome may result from an abnormality in T-cell lymphocyte function. 3. Pathology (1) Minimal-change disease (78%), the glomeruli appear normal. The epithelial cell foot processes fused. More than 95% of children with MCD,and better responding to corticosteroid therapy. (2) Focal sclerosis in glomeruli (6.7%), sclerosis and hyalinosis involving a portion of glom- erular tuft, even only one of the glomeruli, accompanied tubular atrophy. IgM and C3 within sclerotic areas. (3)Mesangial proliferation: Only mesangial proliferation. Immunoglobulin and comp- lement deposits in the mesangial area. (4) Membrane nephrosis: GBM thicker,IC deposits. (5) Membranoproliferative glomerulonephritis: Diffuse proliferation of mesangial cells and mesangial matrix.Electronic density deposits and C3 deposit in mesangial and GBM. 膜增生性肾小球肾炎：系膜细胞和系膜基质增生， 膜增生性肾小球肾炎：系膜细胞和系膜基质增生， 系膜插 入，基底膜双层化 系膜插 入，基底膜双层化 4.Pathophysiology (1) Proteinuria: Massive proteinuria is the most chief characteristics of nephrosis resulti