黄色肉芽肿性肾盂肾炎.ppt

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1、黄色肉芽肿性肾盂肾炎 Introductionl Xanthogranulomatous pyelonephritis(XGP)is an unusual form of chronic pyelonephritis in which the renal parenchyma（肾实质）is destroyed and replaced by lipid-laden foamy macrophages（充满脂质的巨噬细胞）.l It is almost always unilateral(单侧）.l It occurs in 1%of all renal infections.l XGP is

2、4 times more common in females than in men and is usually noted in the fifth and sixth decades of life.l The exact etiology of XGP is unknown,but it is generally accepted that the disease process requires long-term renal obstruction(障碍）and infection.Stones may occur in 75%of patients with XGP but ar

3、e not required to make the diagnosis.l The clinical presentation of patients:lumbar(腰痛）and abdominal pain,weight loss,urinary(泌尿系）symptoms,pale conjunctiva（结膜）,kidney stones and chronic evolution of the disease.High fever and palpable（明显）abdominal mass.l The laboratory,in most cases,revealed anemia,

4、leukocytosis（白细胞增多）very increased ESR,pyuria（脓尿）,hematuria（血尿）and bacteriuria（细菌尿）.pathologyl The pathology on macroscopic examination shows usually a kidney yellow,enlarged in size,and with stones inside.Macroscopic view of the inside of a surgical specimen of a kidney with XP.Presence of multiple

5、cystic cavities.Microscopic examination macrophages are loaded with fat(histiocytes sparkling（闪闪发光的）),explaining his color,in addition to necrosis and infiltration（渗出物）with leukocytes and plasmatic cells histiocytes sparkling grouped and contained lipids,which are called xanthoma（黄色瘤）cells that are

6、associated with a diffuse inflamma-tory infiltrates,where cells are round,the plasmocytes（浆细胞）.foam（泡沫细胞）cells(arrow up),multinucleated(多核）giant(downward arrow)and mononuclear inflammatory infiltrate.Comprehensive imaging manifestation of XGP A 44-year-old woman who reported urinary symptoms for the

7、 past 6 months.Computed tomography was typical for diffuse XGP:enlarged left kidney with a voluminous(大量）central staghorn calculus and hydronephrosis.The renal parenchyma is replaced by multiple confluent fluid-filled masses).imaging manifestation of XGP Diffuse XGP in an 87-year-old man.A post-cont

8、rast enhanced CT image demonstrates diffuse enlargement of the left kidney with massive pelvic dilatation and major caliectasis.imaging manifestation of XGP A 37-year-old woman presented with low back pain and a fever.A post-contrast enhanced CT image demonstrates an enlarged left kidney with hydrop

9、yonephrosis（肾盂积脓），a multilocular intraparenchymatous pseudocyst（多房性的肾实质假囊肿）,inflammatory infiltration（渗出）of the perirenal（肾周）fat,and a thick-walled abscess in the adjacent（邻近的）muscle.imaging manifestation of XGPFocal XGP in a 54-year-old woman.A post-contrast enhanced CT image demonstrates a functio

10、ning left kidney occupied by an ovoid（卵形）exophytic（外生性）mass with necrotic loculations（小腔）and thick walls.Although this is focal XGP,extension of the inflammatory process to the posterior（后方）pararenal space was noted,with local thickening of Gerotas fascia（吉氏筋膜）.Discussionl XGP is difficult to diagnose as the presenting symptoms are non-specific and none of the laboratory findings is diagnostic.