Acute Glomerular Nephritis. Mao Jianhua, Department of Nephrology, The Children Hospital of Zhejiang University,

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1 Acute Glomerular Nephritis Mao Jianhua, Department of Nephrology, The Children Hospital of Zhejiang University,

2 DEFINITION APSGN is a immune-mediated inflammation disease with the mechanism for proliferative glomerulonephritis affecting almost all nephrons in both kidney in patients.

3 EPIDEMIOLOGY APSGN is a disease that affects primarily children, with the peak incidence being between ages of 2 and 12 years, Males are more likely than females to have overt nephritis. Crowded conditions, poor hygiene, malnutrition, and intestinal parasites may results in epidemic outbreaks. APSGN is on the decline in developed countries.

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5 ETIOLOGY Nephritogenic strains of group Aβ hemolytic streptococci: type Ⅻ Other than streptococci, staphylococci, gram-negative rods, and intracellular bacteria also associated with a post-infectious glomerulonephritis recently.

6 Pharyngitis

7 scarlet fever

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9 Impetigo

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11 2014/3/23 毛建华 11

12 PATHOGENESIS Despite the early recognition of an association between streptococcal infection and an acute glomerulonephritis, the pathogenic mechanism of disease remains incompletely understood.

13 PATHOGENESIS 1. Immune complex glomerulonephritis caused by deposition of circulating antigen-antibody complexes. 2. Autoimmune glomerulonephritis caused by deposited IgA being directed against a mesangial self-antigen or neo-antigen. 3. Immune complexes are formed in situ in the mesangium in response to a planted antigen.

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17 PATHOLOGY Endocapillary proliferative nephritis

18 Light microscopy the glomeruli are found to be swollen and filled with cells obscuring much of the delicate network of the normal glomerular tugt.

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22 Immunofluorescence microscopy Granular deposits of IgG and C3 in capillary loops and mesangium

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25 Electron microscopy 1.The proliferation of cells is seen to involve primarily endothelial cells and the mesangium. 2.Electron-dense humplike dense deposits on the epithelial side of the basement membrane.

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28 Clinical Features

29 Latent period: Averages 10 days after a pharyngitis (range 7 to 21 days) May be longer after a skin infection (range 14 to 21 days) Short latent period of less than 7 days presented in patient with?

30 Classically, the syndrome of APSGN presents abruptly with hematuria, edema, azotemia and hypertension. The syndrome can present with an entire spectrum of severity from asymptomatic to oliguric acute renal failure.

31 Classically: 1. oliguria and edema: based on renal fluid & sodium retention. 2. microscopic/gross hematuria 3. hypertension: occurs in more than 75% patients, usually mild to moderate.

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36 In some patients 1. Hypervolemia & congestive heart failure 2. Encephalopathy: confusion, headache, somnolence 3. Oliguric acute renal failure

37 Edema & hypertension typically resolve in 1 to 2 weeks after diuresis, though hematuria and proteinuria may persist for several months, but they usually resolve within 1 year.

38 LABORATORY FEATURE Complement:C 3, CH 50 Blood Renal function examination ESR urinalysis ASO, ADNase-B, Ahase, et al.

39 Dennen P, Douglas I, Anderson R,: Acute Kidney Injury in the Intensive Care Unit: An update and primer for the Intensivist. Critical Care Medicine 2010; 38:

40 Differential diagnosis Rapidly progressive glomerulonephritis Chronic glomerulonephritis Infection-associated glomerulonephritis not caused by streptococcus Secondary glomerulopathies: IgAN

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43 A. 确诊病例 : 需要实验室的确诊依据, 或实验室提示依据 + 临床证据 ; B. 疑似病例 : 仅需要临床证据 ; C. 可能病例 : 仅需要实验室提示依据 ; 实验室确诊依据是指肾活检证实为 APSGN( 内皮细胞增殖, 伴系膜区及沿肾小 球毛细血管袢的颗粒状沉积 [8]); 实验室提示依据包括同时符合以下三条 :1 镜检血尿 : 红细胞 >10 个 / 高倍视野 ; 2 近期链球菌感染的依据 ( 链球菌培养阳性, 或 ASO 升高, 或抗 DNase-B 阳 性 );3 补体 C3 降低 临床证据包括以下四条中至少两条 :1 面部水肿 ;2 中等程度以上的血尿 ;3 高 血压 ;4 外周肢体水肿

44 TREATMENT

45 Treatment of acute PSGN is largely that of supportive care

46 1. Stay in Bed

47 2. DIET CONTROL

48 3. ANTIBIOTICS

49 4. Symptom control diuretics and anti-hypertension therapy

50 1. Congested circulation 2. Encephlopathy 3. Acute renal failure

51 PROGNOSIS

52 Most children (up to 95%) fully recover from APSGN in a matter of weeks or months. In those who do not recover fully, chronic or progressive problems of kidney function may occur. Kidney failure may result in some patients.

53 In some patients, there is evidence that the original diagnosis of APSGN may have been in error, especially for the individuals in whom a renal biopsy was never performed. In these patients, lack of resolution of their renal disease should prompt a renal biopsy to elucidate the underlying cause of glomerular disease other than APSGN.

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55 Acute Post-Streptococcal GN Synonyms: Incidence: Etiology: Clinical: Lab: Path: Clinical Course: Acute proliferative glomerulonephritis, acute post-infectious GN. Peak incidence in children (3-14). Sporatic, mostly winter and spring. Glomerular trapping of circulating antistreptococcal immune complexes. Group A, B-hemolytic streptococci, type 12. Acute nephritic syndrome post-strept pharyngitis or pyoderma. Other infections. Nephritic urine with RBC casts. Evidence of streptococcal infection or serologic evidence of recent infection. Decreased serum complement. Enlarged, hypercellular glomeruli with endothelial and mesangial cell proliferation. Acute inflammation. IgG and C3 in very coarsely granular pattern along GBMs. Discrete, subepithelial hump-like deposits. Children - Excellent prognosis. Adults - Worse prognosis, some develop progressive disease.

56 Post-Streptococcal GN CNS Streptococcal Infection + Strep Assay Hypertension Latent Period Edema Acute Nephritis Proteinuria Hematuria

57 History Present, past and family Physical examination Laboratory Height, weight, blood pressure, optic fundi, presence or absence of abdominal mass, skin appearance, genitalia, edema, complete physical examination. Urinalysis (including microscopic examination & RBC morphology), urine culture, complete blood count, serum electrolytes, creatinine, calcium, serum complement, random urine for total protein, calcium, and creatinine, renal imaging(ultrasound or CT).