<Case Presentation>
A 32-year-old woman suffered from cough with copious whitish
sputum and urine frequency and urgency one week prior to
admission. Besides, she gradually turned lethargic and
disoriented, with complaints of abdomen discomfort, myalgia,
arthralgia, headache, and gingival swelling. One day before
admission, she lost consciousness during urination. She was
sent to local hospital where hypotension and pyuria were
found. The low blood pressure persisted even under intravenous
fluid administration and dopamine infusion, so she was
transferred to intensive care unit of this hospital for
further management.
The patient's family mentioned
that she had had massive postpartum hemorrhage during her
second childbirth 2 years ago and secondary amenorrhea
developed at the age of 30. She recognized to have
light-colored areola, sparse pubic hair, and general malaise
since then.
She had disturbed consciousness at
admission. The temperature was 38.2oC, the pulse was 102 and
the respiration was 24. The blood pressure was 100/60 mmHg.
General physical examination showed no abnormalities except
the pale-looking face, light-colored areola and sparse
axillary and pubic hairs, and slightly decrease deep tendon
reflexes of elbow and knee. Laboratory tests were performed
(Table 1~3).
Table 1. Hematologic laboratory values
on admission
WBC |
RBC |
Hb |
Hct |
MCV |
PLT |
K/μL |
M/μL |
g/dL |
% |
fL |
K/μL |
19.74 |
3.45 |
10.4 |
29.1 |
84.3 |
107 |
Table 2. Blood chemical values on
admission
Alb |
LDH |
BUN |
Cre |
Na |
K |
TG |
T-Cho |
LDL-C |
HDL-C |
g/dL |
U/L |
mg/dL |
mg/dL |
M |
M |
mg/dL |
mg/dL |
mg/dL |
mg/dL |
4.2 |
1176 |
47.8 |
1.3 |
127 |
5.4 |
121 |
89 |
28 |
9 |
Table 3. Endocrinologic
laboratory tests
E2 |
FSH |
LH |
Prolactin |
HGH |
DHEA-S |
Free T4 |
T3 |
TSH |
pg/ml |
mIU/ml |
mIU/ml |
ng/ml |
ng/ml |
μmol/L |
ng/dl |
ng/dl |
μIU/ml |
<20 |
1.9 |
0.7 |
4.2 |
11 |
<0.81 |
0.51 |
40.9 |
5.16 |
|
Cortisol |
ACTH |
|
μg/dL |
pg/ml |
0800hr |
5.2 |
61.9 |
1600hr |
3.1 |
50.6 |
She was treated with
ceftriaxone 1g iv q12h for a total of 7 days. Blood and urine
cultures both yielded E.coli. However, profound hypotension
did not respond to antibiotic, iv fluid and inotropic agents.
Treatment with iv hydrocortisone (100 mg q8h) was begun, under
the impression of adrenal insufficiency, which was later
proved by laboratory tests. The diagnosis of Sheehan's
syndrome was made according to her past history,
endocrinologic examinations and imaging studies (Fig.
1&2). Her blood pressure stabilized after steroid use and
she regained consciousness gradually. She was maintained on
oral prednisolone 7.5 mg per day after discharge from this
hospital.
Fig. 1. Skull X-ray (lateral view): The
sella turcica is intact, No evident destructive bone lesions
or fractures are noted. Frontal sinus is prominent.
Fig.
2. MRI study without and with
enhancement: The anterior pituitary gland was very small in
size and the posterior pituitary and stlak was preserved. The
sellar fossa was filled with CSF and was compatible with empty
sella syndrome or Sheehan's syndrome.
<病案分析 >
本案為一腦下垂體前葉功能不足(anterior pituitary
insufficiency),而以腎上腺危症(adrenal crisis)來表現的病例。在處置上,
如果第一線醫師未想到這個診斷, 可能就會失去治療的契機, 造成患者死亡。故而此病雖罕見, 卻非常重要。在鑑別診斷上,
由於患者一開始的表現常只是疲倦、虛弱等等非特異性的症狀,不容易做早期診斷。 但若同時有低血鈉症與休克的表現,
對輸液與升壓劑反應不佳時, 應立刻想到腎上腺危症之診斷, 並進一步抽血測cortisol 與ACTH以實驗室檢查證實。
在實驗室檢查方面, 早晨8:00 之cortisol level若低於3 μg/dL,可直接診斷為adrenal
insufficiency; 若高於19 μg/dL, 則可排除此診斷。 但若介於這兩者之間, 則必須依賴ACTH
stimulation test做區分: 亦即, 對病人施與250μg之cosyntropin 肌肉注射,
在30及60分鐘後各測一次cortisol level; 若其中有一次到達18μg/dL以上, 就可排除此診斷; 反之,
則可確認病人有adrenal insufficiency。
本案之病人來時是處於所謂adrenal crisis狀態,
此時較為合適的做法, 是抽血測cortisol 及ACTH之後, 立刻給予glucocorticoid
replacement。 可考慮使用dexamethasone, 因為若laboratory
tests無法確定是adrenal insufficiency時, 可以再做ACTH test證實,
而dexamethasone比較不會干擾cortisol之測定。
此外, 病人的病史詢問及理學檢查也是相當重要的。 Secondary
adrenal insufficiency 最常見的原因, 並非本例所見之Sheehan's syndrome,
而是長期使用類固醇抑制本身的hypothalamus-pituitary-adrenal axis,
又因為某些原因停用類固醇時所導致。 此時, 患者外表雖像Cushing's syndrome, 卻有adrenal
insufficiency的表現, 病史上也可問到患者過去吃藥的情況。 此外,
過去是否有接受過腦下垂體手術或腦部放射線治療, 也是相當重要的病史; 而其他axes的腦下垂體機能是否有問題,
如是否合併性腺機能低下、 是否有甲狀腺功能過低的表現, 如怕冷、 便秘、 體重增加等等, 都是檢查患者的重點。
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