A 48-year-old man was sent to the Emergency
Department of a teaching hospital on June 4, 2001
because of drowsy consciousness, nausea, vomiting and
progressive malaise for 3 days. He began to develop
rhinorrhea, cough and sore throat 4 days ago and upper
respiratory tract infection was diagnosed at a local
hospital. He took some oral medications but his symptoms
did not improve.
He had an episode of head injury 15 years ago with
transient loss of consciousness but without significant
neurologic sequelae. He complained of progressive
general malaise and decreased libido in recent one
year.
On examination, his consciousness was drowsy.
The blood pressure was 98/61 mm Hg and pulse rate was 80
per minute. The body temperature was 37.5°C. The
breathing sounds were clear and there were no heart
murmurs. There were scanty hairs over the axillary and
pubic areas, but without hyperpigmentation over the
skin.
The chest X-ray was clear (Figure 1) and the
urinalysis was within normal limits. The biochemistry
data showed hypoglycemia (Glucose: 43 mg/dl) and
hyponatremia (Na: 109.6 mmol/L). Under the suspicion of
adrenal crisis, glucose water, normal saline and
hydrocortisol (100 mg) were administered. His
consciousness level improved and was admitted to the
Endocrinology Ward. The serum levels of testosterone, LH
and FSH were 0.66 ng/ml, 3.3 mIU/ml, and 2.1 mIU/ml,
respectively. The brain MRI showed a normal size of the
sella turcica but the anterior pituitary lobe was small
in size. The signal intensity of the posterior pituitary
lobe was well preserved (Figure 2 and Figure 3),
suggesting the diagnosis of partial empty sella. The TRH
and LHRH tests revealed normal response of TSH and
LH/FSH. Results of the thyroid function (see lab.) were
compatible with hypothyroidism. The titer of
antimicrosomal antibody (AMA) was high (1:20480) and the
thyroid sonography revealed hypoechogenicity of both
lobes. He received replacement therapy of prednisolone,
eltroxin, and testosterone enanthate. He was discharged
on June 21, 2001 in a stable condition.
Laboratory
results:
1. CBC/DC:
|
WBC |
Hb |
Hct |
MCV |
PLT |
Seg. |
Eos. |
Bas. |
Mono. |
Lym. |
|
K/uL |
g/dL |
% |
fL |
K/uL |
% |
% |
% |
% |
% |
June 04 |
5.79 |
9.4 |
26.3 |
59.9 |
256 |
47.5 |
4.1 |
1.2 |
6.4 |
40.5 |
June 19 |
5.48 |
9.9 |
30.0 |
79.8 |
168 |
71.6 |
1.9 |
1.0 |
6.1 |
19.4 | 2.
Biochemistry and electrolyte:
|
BUN |
Cre |
Na |
K |
Ca |
Cl |
P |
Mg |
GOT |
|
mg/dl |
mg/dl |
mmol/l |
mmol/l |
mmol/l |
mmol/l |
mg/dl |
mmol/l |
U/l |
June 05 |
3.3 |
0.5 |
120.0 |
4.4 |
2.1 |
85.0 |
2.8 |
0.76 |
94 |
June 19 |
|
|
133.0 |
4.2 |
|
|
|
|
34 | 3.
U/A:
|
Sp. Gr |
PH |
Pro. |
Glu. |
Ket. |
O.B. |
Urob. |
RBC |
WBC |
Epi. |
Cast |
Bac. |
|
|
|
mg/dl |
g/dl |
|
|
EU/dl |
/HFL |
/HFL |
/HFL |
/LFL |
|
6/5 |
1.023 |
6.0 |
- |
- |
- |
+ |
1.0 |
12-15 |
5-6 |
- |
- |
- |
4. DHEA-SO4 (umol/L): <0.81 (male:
4.6-13.4)
5. hGH (ng/ml): 1.9 (normal: 0.06-5.0)
6. Adrenal function
June 05 |
Cortisol (μg/dl) |
ACTH (pg/nl) |
8 A.M |
39.8 |
<1.0 |
4 P.M |
0.32 |
18.3 | 7.
Thyroid function
June 05 |
FreeT4 (CIA) |
hsTSH (CIA) |
T3 (RIA) |
T4 (RIA) |
Normal range |
ng/dl (0.6-1.75) |
μIU/ml (0.4-4) |
ng/dl (80-200) |
μg/dl (4-12) |
June 05 |
0.49 |
17.5 |
64.2 |
3.76 | 8.
LHRH test
|
0’ |
30’ |
60’ |
120’ |
LH (mIU/ml) |
5.7 |
27.1 |
26 |
20.4 |
FSH (mIU/ml) |
3.3 |
5.8 |
6.4 |
5.9 | 9.
TRH test
|
0’ |
15’ |
30’ |
60’ |
120’ |
TSH (uIU/ml) |
19.7 |
33.7 |
36.7 |
39 |
34.1 |
Prolactin (ng/dl) |
5.4 |
29.3 |
32.1 |
25.1 |
13.2 | 註: Normal
range:
Cortisol |
ACTH |
LH |
FSH |
Testosterone |
μg/dl |
pg/nl |
mIU/ml |
mIU/ml |
ng/ml |
5-25 (8A.M) 2.5-12.5 (4P.M) |
10-65 |
Adult male 1.3-13 |
Adult male 0.9-15 |
Adult
male 3-10 | 本病例為急性腎上腺功能不足,以意識不清和噁心嘔吐來表現。其誘發因素可能為上呼吸道感染。其實驗室檢查十分典型,包括低血糖、低血鈉、嗜伊紅性白血球增加、淋巴球增加。因為病人在急診處已先用類固醇治療,因此影響了隔天cortisol,
ACTH的判讀。病人有性慾減退的症狀,其testosterone 值很低,而且LH, FSH
的值偏低,因此是低性腺促素性腺功能不足 (hypogonadotropin
hypogonadism)。頭部核磁共振發現病人的腦下垂腺前葉較萎縮,但後葉的顯影則正常。所以臨床上的診斷為空鞍症候群合併腦下垂腺功能不足。空鞍
(empty sella) 的形成是由於先天性或後天性蝶鞍膈膜 (diaphrama sellae)
缺損,導致蜘蛛膜下腔的腦脊髓液擴張至蝶鞍中,將腦下垂腺壓扁;或是之前的腦下垂腺腫瘤因血管梗塞而萎縮
(pituitary infarction)。所以空鞍不是完全沒有腦下垂腺組織,而且腦下垂腺柄
(pituitary stalk) 可在核磁共振中清楚顯影 (此稱為infundibulum
sign)。空鞍常常是在頭部影像檢查中意外發現,而空鞍症候群則是指臨床上合併頭痛、視野缺陷、腦脊髓液流涕
(CSF rhinorrhea) 或腦垂腺功能不足。空鞍症候群多發生在中年肥胖女性,有的病患可發現良性顱內高壓
(benign intracranial
hypertension)。治療上是視腦下垂腺功能不足的程度,以標的器官荷爾蒙來補充,譬如類固醇、甲狀腺素、性荷爾蒙。空鞍和腦下垂腺無發育
(pituitary aplasia) 的鑑別診斷主要是靠腦下垂腺後葉在核磁共振上的顯影來區分。
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