下列腎絲球疾病中,何者最易產生新月形(crescent)腎絲球細胞增生?

(A)細基底膜病(thin basement membrane disease)
B)抗基底膜病(anti-basement membrane disease)
(C)瀰漫性膜性腎絲球腎炎(diffuse membranous glomerulonephritis)
(D)糖尿病腎絲球疾病(diabetic glomerular disease)

Ans:(B) 抗基底膜病(anti-basement membrane disease)

詳解:看到crescent formation想到rapid progressive glomerulonephritis (RPGN),故選 B

(A)thin basement membrane disease(thin GBM disease)具家族遺傳性,所以又叫做 familial thin basement membrane disease。既然都說變薄了就不會”增生”,這個選項自然優先刪掉。檢查的話:光學與免疫螢光染色是 normal finding,電子顯微鏡下可看見glomerular basement membrane變薄)。預後不錯,加上是慢性血尿,所以是 benign chronic familial hematuria,較不 會變成 renal failure。

(B)anti GBM disease除了會有crescent鮑氏囊壁層上皮細胞增殖外,還會有IgG的linear deposits。Goodpasture也屬這類,但還另外有肺出血的症狀。

(C)membranous GN病理的表現一開始是會有subepithelial的沉積,接下來會有spike and dome appearance

(D)糖尿病腎絲球疾病(diabetic glomerular disease)腎絲球會有硬化情形,是 nodular sclerosis(eosinophilic)=K-W(Kimmelstiel-Wilson) sclerosis

K-W sclerosis=DM nephropathy

Wire-loop=SLE nephropathy

Crescent formation=RPGN

Linear deposits=Goodpasture syndrome

Humplike deposits=PSGN

Double contour or tram-track=MPGN type 1

Dense ribbon=MPGN type 2


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