A focal skeletal disorder characterized by rapid, disorganized bone remodeling.
Usually occurs after age 40 and most often affects the pelvis, femur, spine, and skull.
Clinical manifestations:
Most patients are asymptomatic.
Bone pain and deformity, degenerative arthritis, pathologic fractures, neurologic deficits due to nerve root or cranial nerve compression(including deafness), and rarely, high output heart failure and osteogenic sarcoma.
Diagnosis:
The radiographic appearance is usually diagnostic, and biopsy is rarely necessary. Serum alkaline phosphatase is elevated. Serum and urine calcium are usually normal.
Indications for therapy:
1. bone pain
2. nerve compression syndromes 3. pathologic fracture
4. elective skeletal surgery 5. progressive skeletal deformity 6. immobilization hypercalcemia 7. hypercalciuria with nephrolithiasis 8. high-output heart failure
9. asymptomatic involvement of weight-bearing bones or the skull 健
Treatment:
Bisphosphonates inhibit excessive bone resorption, relieve symptoms, and restore serum alkaline phosphatase and bone deposition to normal in most patients.
Alendronate 40 mg/day for 6 months, or risedronate 30 mg/day for 2–3 months. The effectiveness of therapy is monitored by measuring serum alkaline phosphatase every 3 months.
Reference:
1. Green GB, Harris IS, Lin GA, Moylan KC. The Washington Manual of Medical Therapeutics. 31
st
Edition 2004; pg: 56-62.
2. Langston AL. Rheumatology(Oxford). 2004;43:955-959.
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腦下垂體腫瘤
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肆、動態內分泌功能試驗及週邊血管、甲狀腺超音波檢查
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(2) Oral glucose tolerance test-growth hormone
* The administratio of oral glucose tolerance test to normal subjects usually results in depression of plasma GH levels. In active acromegalic patients it fails to show such suppression and may demonstrate a rise in GH instead.
Procedure
* Fast overnight is necessary
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婦硬酮ve健t顯e健p酮t餘ent健鐘r餘n鬆健t顯e健面lu錯齡se健s齡lut餘齡n健齡ver健酮健per餘齡鐘健齡陰健up健t齡健慮息健鳴餘nutes幫健健 婦物l齡齡鐘健s酮鳴pl餘n面健酮t健按息層健景息層健業息層健慮扮息層健酮n鐘健慮格息健鳴餘nutes健陰齡r健睪硬健酮n鐘健鉀l齡齡鐘健面lu錯齡se健健 Interpretation
In normal subjects,GH falls to lower than 2 ng/ml or undetectable levels within 30 minutes to 2 hours, though a later rebound of GH levels is common. Acromegaly will demonstrate either no suppression, partial suppression or paradoxical rise (often greater than 5-10 ng/ml). In addition, BS levels show impaired glucose tolerance or
Diabetes range.
(3) TRH stimulation test
*To differentiate hyperthyroxinemia with nonsuppressed TSH due to TSH-secreting pituitary adenoma or thyroid hormone resistance syndrome
*In patients who appear euthyroid ( by clinical data and results of other laboratory tests), but who have eye changes that suggest Gravesophthalmopathy, the TRH test gives a sensitive index of minor aberrations in the pituitary-thyroid axis
Procedure
*TRH 500μg by IV injection and draw TSH at 0,30 and 60 minutes following TRH Interpretation
*TSH rises with an average peak at 20-40minutes(generally an increment of 15μU/ml for adults) and then fall at 60 minutes( less than 2/3 of peak levels). In men over 40, the response is smaller and increment of 2 μU/ml is considered normal.
*Euthyroid Graves’ disease demonstrate blunted response or absent response to TRH .
*TSH secreting adenoma shows no TSH response following TRH injection, while TSH levels increaseas usual in thyroid hormone resistance syndrome
(4) LH-RH stimulation test
Procedure
*Studies in females should preferrably by done in the early follicular phase of menstrual cycle (days 1-7)
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*at least two blood samples should be obtained 15 minutesprior to and immediately 鉀e陰齡re健睪n良硬健酮鐘鳴餘n餘str酮t餘齡n幫健葡顯e健酮ver酮面e健齡陰健t顯e健tw齡健s酮鳴ples健serves健酮s健t顯e健 鉀酮sel餘ne健簡硬健酮n鐘健癇茶硬幫健健
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神nterpret酮t餘齡n健健
葡顯e健簡硬健resp齡nse健t齡健睪n良硬健餘s健usu酮lly健鳴齡re健pr齡n齡un錯e鐘健酮n鐘健餘s健seen健e酮rl餘er健t顯酮n健 t顯e健癇茶硬健resp齡nse幫健神n健n齡r鳴酮l健酮鐘ults層健簡硬健pe酮鬆s健酮re健面ener酮lly健陰齡un鐘健酮t健慮教健t齡健 搬教健鳴餘nutes層健w顯ere酮s健癇茶硬健pe酮鬆s健鳴酮y健齡錯錯ur健l酮ter健餘n健s齡鳴e健p酮t餘ents幫健神n健酮鐘ults層健 簡硬健levels健w餘ll健酮t健le酮st健鐘齡u鉀le健陰齡ll齡w餘n面健睪n良硬健酮鐘鳴餘n餘str酮t餘齡n幫健葡顯e健癇茶硬健r餘se健 餘s健usu酮lly健less健t顯酮n健tw齡陰齡l鐘s健酮n鐘健餘t健餘s健n齡t健unusu酮l健t齡健see健l餘ttle健錯顯酮n面e健餘n健 癇茶硬健levels健even健餘n健n齡r鳴酮l健酮鐘ults幫健理e錯re酮se鐘健resp齡nse健齡陰健pl酮s鳴酮健面齡n酮鐘齡tr齡p餘ns健 鳴酮y健餘n鐘餘錯酮te健e餘t顯er健p餘tu餘t酮ry健鐘餘se酮se健齡r健pr齡l齡n面e鐘健en鐘齡面en齡us健鐘e陰餘錯餘en錯y健齡陰健 睪n良硬幫健
(5) Cortrosyn stimulation test
* To determine the functional capacity of the adrenal cortex by measuring the change in plasma cortisol levels during the administration of ACTH.
*If the patient has suspected symptoms of acute adrenocortical insufficiency, Treatment with dexamethasone 0.25mg administered orally every 8 hours beginning 24 hours prior to the test and continuing for the duration of the test will not interfere with his response of ACTH. The patient should be fed as usual on the day of the ACTH test.
Procedure
*Cortrosyn depot 1mg intramuscularly at time 0 minutes.
*Collect blood for determination of cortisol levels before and at 60 minutes after the injection.
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Interpretation
*Normally, serum cortisol rises to a level greater than 20ug/dl at 60 minutes. Primary adrenal insufficiency showed absent response of serum cortisol level.
*Secondary adrenal insufficiency usually shows blunted response at 60 minutes, but the cortisol level further increase at 24 hours. Occasionally, the secondary adrenal
insufficiency demonstrated normal response at 60 minutes.
(6) Insulin hypoglycemic stimulation test
*To determine growth hormone secretion and the hypothalamic-pituitary-adrenal axis
*The test is contraindicated in patients with old aged, cardiovascular disease or seizure disorder. In suspected hypopituitarism cases( with adrenal insufficiency), the
insulin dosage should be decreased(0.05u/kg) or not to perform this test in these patients.
*Close monitoring of vital signs, S/S of hypoglycemia by doctors is strongly necessary throughout the whole examination.
Procedure
*Regular insulin is given 0.15u/kg intravenously and check the glucose, GH, cortisol at 0, 30, 60, 90, 120 minutes.( Overnight fast before examination)
*If the patient suffered from severe hypoglycemia during examination, the iv glucose should be pushed , but the blood sampling examination should be continued
until 120minutes.( The mild hypoglycemia S/S should also not terminate the test)
*Check the serum glucose, cortisol and GH level at each time points
*A meal should given after test finishment.
Interpretation
*GH usually peaks at 40-90 minutes after insulin injection, and it should increase to an absolute level of 10 ng/ml.Value less than 7 ng/ml is considered abnormal and 7- 10 ng/ml is equivocal. For appropriate stimulation of GH secretion, a fall of BS at least 50% from baseline( i.e. 40mg/dl) is necessary.
*A peak cortisol level greater than 18-20µg/dl implies intact pituitary adrenal response.
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超音波在甲狀腺與副甲狀腺疾病所扮演的角色
甲狀腺與副甲狀腺兩個內分泌腺體，因其位置較為表淺，各式病變容易被超音波 偵測到。據 Semin Roentgenol 2013年一月報導: 題 甲狀腺與副甲狀腺影像學
(作者 J. Lee等人)，該文章主要簡介如何利用超音波診斷甲狀腺與副甲狀腺疾
病。本篇文章影像皆為撰寫作者自己收集之影像資料。
正常甲狀腺解剖構造及超音波影像
甲狀腺是人體最大的內分泌器官，形狀似蝴蝶，其位置主要包覆在頸部環狀軟骨 下方，氣管軟骨之上的氣管兩旁。甲狀腺構造可分為左右兩葉和峽部。兩葉各約 一至二公分厚，二至三公分寬，四公分長。一般右葉比左葉略大，左葉的下方有 食道通過。中間的峽部較小，長約兩公分，只有零點五公分厚。正常成人的甲狀 腺重量介於十五克至二十克間，當甲狀腺腫大（thyroid goiter）時可重達上百克。
甲狀腺因其位置表淺且沒有被骨頭或空氣遮蔽，故可以藉由超音波做清楚且全面 評估。在正常狀態下，因甲狀腺為致密均勻實質器官，所以它的超音波屬於中等 回音，回音強度較週邊肌肉組織高，。超音波横切面时，甲狀腺的兩側可見總頸 動脈及頸靜脈，前方和前側方可見呈低回音的頸前肌群和胸鎖乳突肌。而左葉內 側後方可以看見食道，甲狀腺後方有四個副甲狀腺，但正常情況不易看見。
圖一、正常甲狀腺構造
胸鎖乳突肌 頸前肌群 甲狀腺右葉
正常的甲狀腺為均質的中回音，回音強度較週邊肌肉組織，左葉內下方可見食 道，可請病人吞嚥口水，超音波下可見食道蠕動。
常見甲狀腺疾病與超音波影像
甲狀腺相關疾病包含甲狀腺結節、甲狀腺癌及甲狀腺機能亢進或低下等，臨床上 除了利病史詢問、理學檢查和生化與內分泌功能檢查來加以診斷外，也可以運用 超音波做為輔助之用。以下將甲狀腺超音波於各疾病的特色及運用加以介紹。
甲狀腺結節
結節指的是甲狀腺長出之腫塊，內容物可能包含固體或液體，病理分類上大部分 是良性的組織，只有少數為惡性--即甲狀腺癌。根據文獻統計，用手觸診約有4%
的人可發現有甲狀腺結節，如以超音波評估，則有高達30%至50%的人有甲狀 腺結節，甚至甲狀腺內的結節約二至三釐米即可在超音波下被看到。甲狀腺結節 盛行率可能會隨碘缺乏攝取及年紀上升而增加。一般而言甲狀腺結節多無明顯症 狀，但結節較大時，可能會壓迫氣管、食道、神經而導致呼吸困難、吞嚥困難或 聲音嘶啞等症狀，如結節合併出血時可能會迅速變大而產生局部脹痛不適。大多 的結節沒有伴隨甲狀腺功能異常，但有些會增加甲狀腺素之分泌，產生甲狀腺機 能亢進的臨床症狀，稱為毒性甲狀腺腺瘤（toxic adenoma）。臨床上超音波可幫 助評估結節的大小形狀、邊界、回音、固體或液體組成比例、血流分布和強度及 有無鈣化情形。良性的結節一般邊界清楚、包膜完整、沒有或些微的血流增加，
結節內可能為固體、液體或兩者並存，內容物只有液體多為良性的囊腫（cyst），
囊腫內細微的結晶可能在超音波下呈現彗星狀（comet tail sign）。固體的結節可 能為中或略低之回音，合併囊腫或鈣化的產生。粗鈣化點(macrocalcification)如蛋 型（eggshell）的鈣化，一般不會增加診斷惡性腫瘤之比率。
圖二、甲狀腺囊腫 氣管
食道
甲狀腺左葉 峽部
超音波縱切面下可見甲狀腺右葉有多發性的甲狀腺囊腫，可見囊腫內的膠體 (colloid)呈現彗星狀(comet tail sign)
圖三、良性甲狀腺結節
超音波下可見甲狀腺左葉有四公分邊界清楚、中低回音且周圍血流略為上升之結 節，開刀確認為良性甲狀腺結節。
甲狀腺癌
常見的甲狀腺癌有濾泡癌（papillary carcinoma）、乳突癌（follicular carcinoma）、
髓狀癌（medullary carcinoma）及大多發生於老人家且惡性度高的分化不良癌
（anaplastic carcinoma）。初期甲狀腺癌多無明顯症狀，可能因有淋巴轉移產生 頸部腫塊時才就醫。隨著病情進展，腫塊會侵犯周圍組織時，吞嚥時甲狀腺腫會 移動的程度會減低，甚至可伴隨呼吸與吞嚥困難、聲音嘶啞、頸交感神經節受壓，
甚至是遠端轉移之症狀。惡性腫瘤在一般超音波成像常有以下幾項特色：橫切面 的高度大於寬度（taller than wide），因生長快速導致結節邊界不清楚(irregular
margins)、無完整包膜之影像（absence of halo），腫瘤內部呈現低回音
（hypoechoic）、與乳突癌相關之小鈣化點（microcalcification）。另外也可以評 估有無頸部淋巴結腫大及對週邊構造之壓迫或侵犯程度。若搭配杜普勒超音波
（Doppler ultrasound）則可觀察是否有結節內血流增加的情形（increased internal or central flow）。因惡性細胞沒有正常細胞之規則排列，且腫瘤細胞較為緻密，
所以有研究利用彈性超音波（elastography），對結節的軟硬度進行評估，提高 超音波對良惡性結節的診斷力。