内科学教学课件：Nephrotic syndrome
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1.Minimal change disease:
• MCD is commonly seen in children but occasionally present in adult.
• Male predominance
• Symptoms: NS, rarely cause acute renal failure, blood pressure is normal ,GFR is normal or slightly reduced , 15%MCD show microscopic hematuria;
• 分型： 经典型 塌陷型 顶端型 细胞型 非特殊型
【pathology & clinical features】
• NS.80% patients have microscopic hematuria, 50% have hypertention, decreased renal function is present in 30%
※Chapter 4 Nephrotic syndrome
(NS)
Diagnosis standard:
1.Protein in urine>3.5g/d
2.Serum albumin<30g/l
3.Edema
4.Blood lipid rise Essentials of diagnosis include proteinuria
【pathology & clinical features 】
• a characteristic fusion of epithelial foot processes
• Treatment: Spontaneous alleviate occurs in 30%-40% of patients; Sensitive to glucocortical hormone; MCD is a highly steroid-responsive and carries an excellent prognosis.
【pathophysiology】
• Large amount of proteinuria:
molecular & charge barrier are damaged → glomerular proteinuria
• Changes of plasma protein:
①ALB synthesis↑<lose↑+ catabolism ↑; pro. intake↓≥ pro. in plasma↓
Pictures of ← Minimal Change
Disease ↙↓
电镜观察 左：正常肾小球足突 右：肾小球足突广泛融合
【pathology & clinical features】
2.Focal segmental glomerular sclerosis (FSGS):
• Adolescence male
(FSGS) 3.Membranous nephropathy 4.Mesangial proliferative
glomerulaonephritis 5.Membranoproliferative
glomerulonephritis
【pathology & clinical features】
2.secondary～: purpura nephritis, hepatitis B virus related nephritis, lupus nephritis, diabetic nephropathy,
renal amyloidosis, myeloma nephropathy, lymphoma/noumenal tumor nephropathy, etc
• Light microcopy shows the lesions of focal segmental glomerular sclerosis. IgM and C3 are seen in the sclerotic lesions on immunofluorescence .Electron microcopy shows fusion of epithelial foot processes as seen in minimal change disease.
② other kinds of pro↓→ infection, hypercoagulability, immune, endocrine disturbance, trace elements↓
【pathophysiology】
• Edema: hypoproteinuria→ intravascular colloid osmotic p. ↓→ edema
分 类
儿童
青少年
Baidu Nhomakorabea
中老年
微小病变型肾病 系膜增生性肾小球肾炎 膜性肾病
原
发
系膜毛细血管性肾小球 肾炎
性
局灶节段性肾小球硬化
过敏性紫癜性肾 系统性红斑狼疮
炎
继
乙肝病毒相关性 过敏性紫癜性肾炎 肾小球肾炎
糖尿病肾病 肾淀粉样变性
发 系统性红斑狼疮 乙肝病毒相关性肾小球 骨髓瘤性肾病
肾炎
性
淋巴瘤或实体肿 瘤性肾病
• Hyperlipidemia: lipoprotein synthesis↑+ lipoprotein break up↓ → cholesterol, triglyceride↑
Pathological types
1.Minimal change disease (MCD) 2.Focal segmental glomerular sclerosis
• Treatment: 30%-50% of patients treat with glucocorticoids for 8 weeks. Up to70% respond when steroid therapy is prolonged for 3－4months. Mycophenolate mofetil is another option in resistant cases
of >3.5g/1.73m2.24h, hypoalbuminemia (serum albumin <30g/l)
病因
肾
原发性
病
由多种不同病理类型
综
合 征
继发性
的肾小球疾病引起
【pathogeny】
1.primary～: pathogeny isn’t clear in young persons
• MCD is commonly seen in children but occasionally present in adult.
• Male predominance
• Symptoms: NS, rarely cause acute renal failure, blood pressure is normal ,GFR is normal or slightly reduced , 15%MCD show microscopic hematuria;
• 分型： 经典型 塌陷型 顶端型 细胞型 非特殊型
【pathology & clinical features】
• NS.80% patients have microscopic hematuria, 50% have hypertention, decreased renal function is present in 30%
※Chapter 4 Nephrotic syndrome
(NS)
Diagnosis standard:
1.Protein in urine>3.5g/d
2.Serum albumin<30g/l
3.Edema
4.Blood lipid rise Essentials of diagnosis include proteinuria
【pathology & clinical features 】
• a characteristic fusion of epithelial foot processes
• Treatment: Spontaneous alleviate occurs in 30%-40% of patients; Sensitive to glucocortical hormone; MCD is a highly steroid-responsive and carries an excellent prognosis.
【pathophysiology】
• Large amount of proteinuria:
molecular & charge barrier are damaged → glomerular proteinuria
• Changes of plasma protein:
①ALB synthesis↑<lose↑+ catabolism ↑; pro. intake↓≥ pro. in plasma↓
Pictures of ← Minimal Change
Disease ↙↓
电镜观察 左：正常肾小球足突 右：肾小球足突广泛融合
【pathology & clinical features】
2.Focal segmental glomerular sclerosis (FSGS):
• Adolescence male
(FSGS) 3.Membranous nephropathy 4.Mesangial proliferative
glomerulaonephritis 5.Membranoproliferative
glomerulonephritis
【pathology & clinical features】
2.secondary～: purpura nephritis, hepatitis B virus related nephritis, lupus nephritis, diabetic nephropathy,
renal amyloidosis, myeloma nephropathy, lymphoma/noumenal tumor nephropathy, etc
• Light microcopy shows the lesions of focal segmental glomerular sclerosis. IgM and C3 are seen in the sclerotic lesions on immunofluorescence .Electron microcopy shows fusion of epithelial foot processes as seen in minimal change disease.
② other kinds of pro↓→ infection, hypercoagulability, immune, endocrine disturbance, trace elements↓
【pathophysiology】
• Edema: hypoproteinuria→ intravascular colloid osmotic p. ↓→ edema
分 类
儿童
青少年
Baidu Nhomakorabea
中老年
微小病变型肾病 系膜增生性肾小球肾炎 膜性肾病
原
发
系膜毛细血管性肾小球 肾炎
性
局灶节段性肾小球硬化
过敏性紫癜性肾 系统性红斑狼疮
炎
继
乙肝病毒相关性 过敏性紫癜性肾炎 肾小球肾炎
糖尿病肾病 肾淀粉样变性
发 系统性红斑狼疮 乙肝病毒相关性肾小球 骨髓瘤性肾病
肾炎
性
淋巴瘤或实体肿 瘤性肾病
• Hyperlipidemia: lipoprotein synthesis↑+ lipoprotein break up↓ → cholesterol, triglyceride↑
Pathological types
1.Minimal change disease (MCD) 2.Focal segmental glomerular sclerosis
• Treatment: 30%-50% of patients treat with glucocorticoids for 8 weeks. Up to70% respond when steroid therapy is prolonged for 3－4months. Mycophenolate mofetil is another option in resistant cases
of >3.5g/1.73m2.24h, hypoalbuminemia (serum albumin <30g/l)
病因
肾
原发性
病
由多种不同病理类型
综
合 征
继发性
的肾小球疾病引起
【pathogeny】
1.primary～: pathogeny isn’t clear in young persons