< Case History >
A 23-year-old male was a
previously healthy college boy. Decreased urine amount,
bilateral lower legs edema, and body weight gain developed
since one week ago. Routine urinalysis at OPD showed RBC: 5-10
per high power field and protein (+++). Under the impression
of nephrotic syndrome, he was admitted for further evaluation
and renal biopsy.
At admission, physical examination
revealed regular pulse with 80/minute, BP 140/94 mmHg, and
body weight 70 Kg. Nothing specific was found during physical
examination except moderate degree of bilateral lower legs
pitting edema. Laboratory examinations showed: prothrombin
time and activated partial thromboplastin time were within
normal limits. Plasma electrolytes levels were normal, but
urine sodium was only 7 mEq/l. Urinalysis showed severe
proteinuria and microscopic hematuria with dysmorphic changes.
His creatinine clearance was only 9.8 ml/min and his daily
protein loss was 10.1 grams. Ultrasonography revealed enlarged
bilateral kidneys with increased echogenecity.
However, progressive dyspnea
with oliguria was noted after an operation for a peritonitis
episode, and marked pulmonary congestion progressed despite of
the use of diuretics, which made temporary hemodialysis
necessary. Severe dyspnea developed after two smooth sessions
of hemodialysis. CPR and mechanical ventilation with 100%
oxygen were given immediately. His arterial blood gas was: pH
7.325, PaCO2 28.2 mmHg, PaO2 42.9 mmHg, HCO3- 14.3 mEq/l, and
O2
saturation 76%. Pulmonary embolism
was highly suspected because of the great arterial-alveolar difference
in oxygen pressure gradient (634.8 mmHg; normal <
15 mmHg). Serum fibrinogen was 1308 mg/dl (normal 151~375
mg/dl). D-dimer was negative. Heparinization using low-molecular-weight heparin
(LMWH) was given subcutaneously three times per day. He
was also treated with methyl-prednisolone, 500 mg twice a day
for three days followed by oral 1 mg/Kg/day
prednisolone. His renal function deteriorated due to the unsteady
cardio-pulmonary condition, and continuous arterio-venous hemodialysis was given.
99mTc-MAA/ DTPA aerosol lung
perfusion & ventilation scan revealed significant
segmental perfusion defects in the posterior basal segment of
right lung. MRA revealed complete obliteration of right
superior pulmonary artery and focal filling defect of right
inferior pulmonary artery with decreased vascularity over
right lower lung (Fig. 1 ). His respiratory condition
was stable there after and renal biopsy was done. He was
discharged and followed as an outpatient, treated with 1
mg/Kg/day prednisolone and 2.5 mg/day warfarin. MRA was
performed 5 months later, and it revealed completed remission.
(Fig.2
)
< Laboratory Data
>
項目 |
WBC |
RBC |
Hb |
Hct |
MCV |
PLT |
單位 |
K/μL |
M/μL |
g/dL |
% |
fL |
K/μL |
2/13 |
6.28 |
5.32 |
14.7 |
43.7 |
89.2 |
322 |
項目 |
Alb |
GOT |
GPT |
Chol |
TG |
BUN |
Crea |
UA |
單位 |
g/dL |
IU/L |
IU/L |
mg/dL |
mg/dL |
mg/dL |
mg/dL |
mg/dL |
2/13 |
2.03 |
24 |
28 |
365 |
95 |
37 |
1.6 |
9.0 |
2/19 |
|
|
|
|
|
95 |
3.5 |
|
8/29 |
4.4 |
|
|
|
|
18 |
1.2 |
|
項目 |
IgG |
IgA |
IgM |
C3 |
C4 |
ANA |
單位 |
mg/dL |
mg/dL |
mg/dL |
mg/dL |
mg/dL |
|
|
107 |
136 |
87.3 |
102 |
23.1 |
Neg. |
項目 |
Fibrinogen |
3-P |
D-dimer |
單位 |
mg/dL |
|
μg/mL |
參考值 |
151~375 |
Negative |
<0.5 |
檢驗值 |
1308 |
1:10 |
- |
病例分析
此位23歲男性一開始以腎病症候群的最常見症狀-水腫來表現,故住院安排腎臟穿刺檢查。住院中之生化檢驗發現該病患合併有腎病症候群常見的低白蛋白血症及高脂血症,而且腎功能也不正常。此病患在住院隔天因突發的腹膜炎而接受開刀及輸液,導致急性的水分累積,造成肺水腫情形,腎功能亦更加惡化,故接受兩次的臨時性血液透析治療移除多餘水分,以緩解症狀。
該病患於病情穩定之後,不幸又突發呼吸困難及hypoxia的情形,並因呼吸衰竭而使用呼吸器輔助治療。經評估血氧指標,發現有很高的
P(A-a) O2,故懷疑有肺栓塞情形,後來並經過Ventilation-perfusion lung scan
及核磁共振MRA的影像學證實。其治療方式是使用抗凝血劑治療,但因該病患接受手術後不久,且曾有短暫性上消化道出血情形,故使用較不具出血傾向的低分子量肝素治療,治療效果顯著。而腎病症候群的部分於嚴重期使用
pulse steroid
治療,而後並須口服Prednisolone一段時間,並慢慢減量,最後也達成治療目標-沒有尿蛋白、腎功能好轉。當患者對類固醇反應不佳時,可能需合併使用免疫抑制劑,如cyclophosphamide等。
肺栓塞常發生於下肢靜脈病變、癌症、心衰竭、長期臥床、近期大手術後之患者,而孕婦及使用口服避孕藥女性也是高危險群。腎病症候群患者因腎臟流失大量蛋白質而導致肝臟加速製造一系列蛋白,而促使一些分子量較大的凝血因子增加,進而造成腎病症候群患者的這種易凝血及栓塞的狀態。最常見的情形是下肢深部靜脈栓塞,腎靜脈栓塞及肺栓塞也不算少見。
|