2010年6月26日 星期六

蛋白尿(proteinuria)和腎臟切片(renal biopsy) (局部性腎絲球硬化症Focal segmental glomerulosclerosis, FSGS)

一位洗腎年輕患者的弟弟,國中年紀,也是一樣有蛋白尿(total protein loss > 5 gm/day)合併血清肌酸酐(creatinine 2.0 mg/dl)過高。以下是他的尿液鏡檢報告:
檢體:U

COLOR                    Yellow
TURBIDITY                Clear
SP.GRAVITY                1.013
PH                        6.5
LEUKOCYTE       cell/uL  Negative
NITRITE         mg/dL    Negative
PROTEIN         mg/dL    3+ (300) ** mg/dl
GLUCOSE         mg/dL    Negative
KETONE          mg/dL    Negative
UBG             EU/dL    0.1
BILIRUBIN       mg/dL    Negative
BLOOD           cell/uL  3+ (200)  ** cell/ul
RBC             /uL      305       ** /ul
WBC             /uL      0
SQUAMUS         /uL      0

眼見自己念高職的大兒子己經在洗腎,看到二兒子的抽血報告,媽媽心中衝擊之大,可想而知。一開始二兒子在別診所追蹤,媽媽後來問我腎臟切片是否一定要做?我的回答是當然,因為一般的自體免疫抗體檢查都找不出病因,也沒有藥物或是感染因素在其中,當然要做腎臟切片,看是否有特殊病因?才能使用免疫抑制劑來對症治療。

腎臟切片報告:局部性腎絲球硬化症(Focal segmental glomerulosclerosis, FSGS)
以下是FSGS在H&E stain
 
這是一個難治的腎臟疾病,需要好好的配合醫療,才能避免掉將來的腎臟衰竭。想到這年輕人,還有他己經在洗腎的哥哥(並沒有做腎臟切片,所以不知原發病因),FSGS少數是因有家族性基因的異常(杜克大學基因研究)。他們兄弟倆是否是同一病?現在己經不可考了,只是希望這弟弟的病情能得到控制而穩定下來。

附上腎臟切片H&E STAIN、免疫螢光和電子顯微鏡報告
DX:
   KIDNEY, BIOPSY
     ---- FOCAL SEGMENTAL GLOMERULOSCLEROSIS, CONSISTENT WITH

ADDENDUM:
   IF STUDY (IF2010=48)
     THERE ARE 2 GLOMERULI ON THE IMMUNOFLUORESCENCE STUDY SECTIONS.

                             CAPILLARY LOOP            MESANGIUM
     IgG                         (-)                      (-)
     IgA                         (-)                      (-)
     IgM                         (1+, G, F)               (1+, G, F)
     C3                          (1+, G, F)               (-)
     C4                          (-)                      (-)
     C1q                         (-)                      (-)
     FIBRINOGEN                  (2+, L, S)               (-)
     KAPPA:                      (-)                      (-)
     LAMBDA:                     (-)                      (-)

GROSS D:
   THE SPECIMEN SUBMITTED CONSISTS OF 3 CORES OF RENAL TISSUE FRAGMENTS
   MEASURING UP TO 1.0 CM IN LENGTH. THEY ARE DIVIDED INTO 3 PARTS. ONE IS
   FROZEN FOR IMMUNOFLUORESCENCE STUDY (0.5 CM IN LENGTH); ONE IS FIXED IN
   GLUTARALDEHYDE FOR ELECTRON MICROSCOPIC STUDY (0.2 CM IN LENGTH); THE
REST IS FIXED IN FORMALIN FOR ROUTINE H&E STAIN AND OTHER SPECIAL STAINS.

MICRO D:
   THERE ARE 2-5 GLOMERULI ON THE SECTION.  NONE OF THEM IS COMPLETELY
   SCLEROTIC.
   GLOMERULI:
     1. CAPILLARY LOOPS:
        a. MOST ARE DELICATE AND THIN
        b. IRREGULAR THICKENING IN FOCAL AREA
        c. PAM STAIN: HETEROGENEOUS STAINING
     2. MESANGIUM:
        a. MESANGIAL CELL PROLIFERATION: PRESENT OCCASIONALLY
        b. MESANGIAL MATRIX: SLIGHTLY INCREASED
        c. PAS STAIN: NEUTROPHIL: ABSENT
     3. LOBULAR ARCHITECTURE: EXAGGERATED, FOCALLY
     4. SEGMENTAL PROLIFERATING AND SCLEROSING LESIONS ARE IDENTIFIED
        IN ONE GLOMERULUS WITH ATTACHMENT OT THE BOWMAN'S CAPSULE.
     5. CRESCENT: ABSENT
   TUBULES:
     1. VACUOLIZATION OF THE EPITHELIUM: PRESENT OCCASIONALLY
     2. ATROPHY: PRESENT (30 % OF THE TUBULES)
     3. NECROSIS OF EPITHELIUM: PRESENT OCCASIONALLY
     4. THYROIDIZATION: ABSENT
     5. CASTS: HYALINE
   INTERSTITIUM:
     1. FIBROSIS: SEVERE (50% OF THE INTERSTITIUM)
     2. ACUTE AND CHRONIC INFLAMMATORY CELL INFILTRATION:
        SEVERE (50% OF THE INTERSTITIUM)
     3. FOAMY CELLS: PRESENT FOCALLY
   VESSELS:
     1. UNREMARKABLE

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