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<Case Presentation
>
A 62 y/o man was admitted to our
hospital due to fever and abdominal pain noted for 2 days. The
patient was diagnosed to have hypertension and autosomal
dominant polycystic kidney disease (ADPKD) about 12 years ago
and had been regularly treated at a local hospital since then.
Occasional abdominal distension and flank pain were
experienced these few years. Two days before admission, he
started to suffer from chills and fever. Progressive abdominal
distension as well as intermittent dull abdominal pain were
also noted. He took some drugs for fever and pain by himself,
but the above symptoms persisted for 2 days. As nausea and
vomiting occurred the third day with aggravated abdominal
pain, he was sent to our emergency department for further
management.
On arrival, the patient looked
weak and irritable. The body temperature was 39.5 ℃, the pulse
rate was 96 beats per minute, and the respiration rate was 22
per minute. His blood pressure was 182/100 mmHg. His abdomen
was markedly distended with diffuse tenderness. Right flank
knocking tenderness was also found. His skin was a little dry.
Other physical examination was not significant. His urine
looked clear and urinalysis showed only mild microscopic
hematuria. Tracing back his history, he had no cough,
diarrhea, urinary frequency or dysuria.
Table 1. Hematologic Laboratory Values.
| RBC |
Hb |
Hct |
MCV |
PLT |
WBC |
Seg. |
| M/μL |
g/dL |
% |
fL |
K/μL |
K/μL |
% |
| 4.55 |
14.6 |
41.5 |
88.9 |
116 |
12.1 |
89 |
Table 2. Blood Chemical Values.
| Alb |
Glo |
Sugar |
Bil (T) |
GOT |
GPT |
BUN |
Cr |
| g/dL |
g/dL |
mg/dL |
mg/dL |
U/L |
U/L |
mg/dL |
mg/dL |
| 3.7 |
3.5 |
106 |
0.7 |
59 |
63 |
66.8 |
2.4 |
| P |
Na |
K |
Ca |
Cl |
| mg/dL |
mmol/L |
mmol/L |
mg/dL |
mmol/L |
| 4.9 |
148 |
3.8 |
10.0 |
110
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As the
patient had vomiting, high fever, azotemia and mild
hypernatremia, normal saline and 2.5% glucose-saline was given
to maintain adequate intravascular volume. His high blood
pressure was treated with anti-hypertensive drugs. Abdominal
echosonography revealed bilateral enlarged kidneys with
numerous renal cysts of different sizes. A few small cysts
were also found in his liver and his pancreas. There was no
renal abscess noted, and the cyst content appeared to be
clear. Supine chest film had no significant finding. Empirical
antibiotic with cefoxitine was therefore given for possible
sepsis and intra-abdominal infection. However, his fever
persisted and the abdominal pain became more severe on the
second hospital day, so magnetic resonance image (MRI) was
arranged which showed abnormal signal inside some renal cysts.
Blood culture and urine culture were both negative. Under the
impression of renal cyst infection, antibiotic was shifted to
ciprofloxacin, and afterwhich fever and abdominal pain
improved gradually.
<病案分析>
本病人為顯性遺傳多囊腎(ADPKD)合併高血壓患者，因局部囊腫感染引起發燒、嘔吐、腹脹及腹痛等症狀。ADPKD是一種常見的腎臟遺傳疾病，盛行率在各國約為1:400至1:1000不等；主要病變為兩側腎臟變大且佈滿大小不一的囊腫，並造成腎臟功能漸漸衰退。臨床表現包括:
(一) 腰痛和血尿 – 可能原因有囊腫出血、囊腫發炎或腎臟結石； (二) 腹脹及食慾不振 –
主要是因為兩側腎臟越長越大並壓迫腸胃所致； (三) 高血壓； (四) 慢性腎衰竭或末期腎病； (五)
肝臟囊腫合併肝功能異常； (六) 心臟二尖瓣脫垂； (七) 顱內動脈瘤破裂造成顱內出血甚至死亡。
其他可能會有囊腫發生的地方尚有胰臟、脾臟、卵巢、睪丸及攝護腺等，但一般不會造成嚴重問題。本病人有腰痛、血尿、腹脹、高血壓、肝和腎功能異常等症狀，也有肝臟及胰臟的囊腫，是多囊腎疾病的典型案例。
本病人不幸發生了囊腫感染，一般除了有腹脹、腹痛、發燒、白血球升高等症狀外，在超音波檢查有時候會看到部份腫囊中之囊液有混濁變化。本病人因超音波檢查無明顯囊腫感染跡象，為確定診斷及排除其他腹腔內感染，故安排核磁共振檢查，並發現有多顆囊腫發炎。治療方面，一般用來治療尿道感染的抗生素無法穿透腎臟囊腫，所以必須選擇可以穿透囊腫的抗生素，如：ciprofloxacin或trimethoprim
-sulfamethoxazole等，療程視情況而定，但通常需長達六週或以上。至於多囊腎致腎衰竭方面，只能靠透析治療或腎臟移植，目前沒有根治的方法。
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Case Discussion
繼續教育考題