《垂體前葉功能減退》ppt課件

1、垂體前葉功能減退垂體前葉功能減退解放軍總醫(yī)院內(nèi)分泌科解放軍總醫(yī)院內(nèi)分泌科竇京濤竇京濤垂體解剖 Functions of the pituitary gland: Each lobe of the pituitary gland produces certain hormones. anterior lobe: growth hormone prolactin - to stimulate milk production after giving birth ACTH (adrenocorticotropic hormone) - to stimulate the adrenal glands

2、TSH (thyroid-stimulating hormone) - to stimulate the thyroid gland FSH (follicle-stimulating hormone) - to stimulate the ovaries and testes LH (luteinizing hormone) - to stimulate the ovaries or testes intermediate lobe: melanocyte-stimulating hormone - to control skin pigmentation posterior lobe: A

3、DH (antidiuretic hormone) - to increase absorption of water into the bloodby the kidneys oxytocin - to contract the uterus during childbirth and stimulate milk production Hypopituitarism Hypopituitarism is a general term that refers to any under function of the pituitary gland. This is a clinical de

4、finition used by endocrinologists and is interpreted to mean that one or more functions of the pituitary are deficient. The term may refer to both anterior and posterior pituitary gland failure. Causes of hypopituitarism Deficient pituitary gland function can result from damage to either the pituita

5、ry or the area just above the pituitary, the hypothalamus. The hypothalamus contains releasing and inhibitory hormones which control the pituitary. Since these hormones are necessary for normal pituitary function, damage to the hypothalamus can also result in deficient pituitary gland function. Inju

6、ry to the pituitary can occur from a variety of insults, including damage from an enlarging pituitary tumor, irradiation to the pituitary, pituitary apoplexy, trauma and abnormal iron storage (hemochromatosis). With increasing damage there is a progressive decrease in function. There appears to be a

7、 predictable loss of hormonal function with increasing damage. The progression from most vulnerable to least vulnerable is usually as follows: first is growth hormone (GH), next the gonadotropins (LH and FSH which control sexual/reproductive function), followed by TSH (which control thyroid hormone

8、release) and finally the last to be lost is typically ACTH (which controls adrenal function). Sheehans Syndrome Sheehans syndrome is a condition that may occur in a woman who has a severe uterine hemorrhage during childbirth. The resulting severe blood loss causes tissue death in her pituitary gland

9、 and leads to hypopituitarism following the birth. Deficiency of ACTH and cortisol Deficiency of ACTH resulting in cortisol deficiency is the most dangerous and life threatening of the hormonal deficiency syndromes. With gradual onset of deficiency over days or weeks, symptoms are often vague and ma

10、y include weight loss, fatigue, weakness, depression, apathy, nausea, vomiting, anorexia and hyperpigmentation. As the deficiency becomes more serious or has a more rapid onset, (Addisonian crisis) symptoms may include confusion, stupor, psychosis, abnormal electrolytes (low serum sodium, elevated s

11、erum potassium), and vascular collapse (low blood pressure and shock) which can be fatal. Treatment consists of cortisol administration or another similar steroid (like prednisone). For patients with acute adrenal insufficiency (Addisonian crisis), rapid intravenous administration of high dose stero

12、ids is essential to reverse the crisis.Deficiency of TSH and thyroid hormoneDeficiency of thyroid hormone causes a syndrome consisting of decreased energy, increased need to sleep, intolerance of cold (inability to stay warm), dry skin, constipation, muscle aching and decreased mental functions. Thi

13、s constellation of symptoms is very uncomfortable and is often the symptom complex that drives patients with pituitary disease to seek medical attention. Replacement therapy consists of a daily pill called thyroxine (Synthroid, Levothyroxine etc). The correct dose is determined through blood tests.D

14、eficiency of LH and FSH (Hypogonadotropic Hypogonadism)Women develop ovarian suppression with irregular periods or absence of periods (amenorrhea), infertility, decreased libido, decreased vaginal secretions, breast atrophy, and osteoporosis. Blood levels of estradiol are low. Estrogen should be rep

15、laced and can be given orally as Premarin or estrace, or can be given as a patch applied twice weekly. Women taking estrogen also need to take progesterone replacement (unless they have undergone a hysterectomy). Annual pap smears and mammograms are mandatory.Men develop testicular suppression with

16、decreased libido, impotence, decreased ejaculate volume, loss of body and facial hair, weakness, fatigue and often anemia. On testing, blood levels of testosterone are low and should be replaced. In the United States, testosterone may be given as a bi-weekly intramuscular injection, a patch form, or

17、 a gel preparation. In other countries, oral preparations of testosterone are available. Growth Hormone DeficiencyGrowth hormone is necessary in children for growth, but also appears necessary in adults to maintain normal body composition (muscle and bone mass). It may also be helpful for maintainin

18、g an adequate energy level, optimal cardiovascular status and some mental functions. Symptoms of GH deficiency in adults include fatigue, poor exercise performance and symptoms of social isolation. GH is only available in injectable form and must be given 6-7 times per week.Antidiuretic Hormone defi

19、ciency causing diabetes insipidusThis problem arises from damage to the pituitary stalk or the posterior pituitary gland. It may occur transiently after transsphenoidal surgery but is rarely permanent. Patients with diabetes insipidus have increased thirst and urination. Replacement of antidiuretic

20、hormone resolves these symptoms. Antidiuretic hormone (ADH) is currently replaced by administration of DDAVP (also called Desmopressin) a synthetic type of ADH. DDAVP can be given by subcutaneous injection, intranasal spray, or by tablet, usually once or twice a day. 主要緣由主要緣由一、原發(fā)一、原發(fā)、垂體缺血性壞死：產(chǎn)后大出血、垂

21、體缺血性壞死：產(chǎn)后大出血Sheehan)綜合征、糖尿病、顳綜合征、糖尿病、顳動(dòng)脈炎、子癲等動(dòng)脈炎、子癲等、垂體區(qū)腫瘤：原發(fā)于鞍內(nèi)的腫瘤，如嫌色細(xì)胞瘤、顱咽管瘤；、垂體區(qū)腫瘤：原發(fā)于鞍內(nèi)的腫瘤，如嫌色細(xì)胞瘤、顱咽管瘤；鞍旁腫瘤：腦膜瘤、視神經(jīng)膠質(zhì)瘤。鞍旁腫瘤：腦膜瘤、視神經(jīng)膠質(zhì)瘤。、垂體卒中：普通與垂體瘤有關(guān)、垂體卒中：普通與垂體瘤有關(guān)、醫(yī)源性鼻煙部或蝶鞍區(qū)放射治療后、手術(shù)創(chuàng)傷毀壞、醫(yī)源性鼻煙部或蝶鞍區(qū)放射治療后、手術(shù)創(chuàng)傷毀壞、免疫性疾病、免疫性疾病、感染性疾病、感染性疾病、海綿竇血栓構(gòu)成及原發(fā)性空泡蝶鞍、海綿竇血栓構(gòu)成及原發(fā)性空泡蝶鞍主要緣由繼發(fā)性、垂體柄破壞性外傷、腫瘤或動(dòng)脈瘤壓迫及手術(shù)創(chuàng)傷

22、、下丘腦或其他中樞神經(jīng)系統(tǒng)病變創(chuàng)傷、惡性腫瘤、類肉瘤、異位松果體瘤及神經(jīng)性厭食等機(jī)理 缺血缺血 破壞破壞臨床表現(xiàn)臨床表現(xiàn)一、垂體前葉機(jī)能減退一、垂體前葉機(jī)能減退1、PRL和和GH分泌缺乏：分泌缺乏： PRL和和GH分泌缺乏是產(chǎn)后大出血最先出現(xiàn)分泌缺乏是產(chǎn)后大出血最先出現(xiàn) 的垂體激素分泌受損表現(xiàn)的垂體激素分泌受損表現(xiàn) PRL 產(chǎn)后無乳、乳房不脹產(chǎn)后無乳、乳房不脹 GH 血糖血糖 2、促性腺激素缺乏：產(chǎn)后閉經(jīng)、性欲減退、陰毛零落、乳房萎縮、促性腺激素缺乏：產(chǎn)后閉經(jīng)、性欲減退、陰毛零落、乳房萎縮及內(nèi)及內(nèi) 外生殖器萎縮和不育外生殖器萎縮和不育 臨床表現(xiàn)促甲狀腺激素分泌缺乏促甲狀腺激素分泌缺乏:表情冷淡

23、、反響愚鈍、怕冷、健表情冷淡、反響愚鈍、怕冷、健 忘、面色慘白、眉毛頭發(fā)稀少、忘、面色慘白、眉毛頭發(fā)稀少、 心率慢、可有或無黏液性水腫心率慢、可有或無黏液性水腫促腎上腺皮質(zhì)激素分泌缺乏：無力、食欲不振、不耐饑促腎上腺皮質(zhì)激素分泌缺乏：無力、食欲不振、不耐饑 餓、體重減輕、心界減少、心音餓、體重減輕、心界減少、心音 低、血壓低、抵抗力差低、血壓低、抵抗力差危象危象危象前期：危象前期： 某些誘因作用某些誘因作用 精神、神志病癥：精神、神志病癥： 嚴(yán)重脆弱無力、精神萎靡、表情冷淡、嗜睡、嚴(yán)重脆弱無力、精神萎靡、表情冷淡、嗜睡、 消化系統(tǒng)病癥：消化系統(tǒng)病癥： 厭食、惡心、嘔吐，進(jìn)食或飲水既吐，合并中上

24、腹痛，繼續(xù)厭食、惡心、嘔吐，進(jìn)食或飲水既吐，合并中上腹痛，繼續(xù)2-4周周 消瘦、脫水消瘦、脫水 心血管系統(tǒng)：心血管系統(tǒng)： 收縮壓收縮壓80-90mmHg，脈壓差小，體位性低血壓，脈壓差小，體位性低血壓 體溫正?；蚋邿狍w溫正?；蚋邿?高熱伴惡心，嘔吐，短時(shí)進(jìn)入危象高熱伴惡心，嘔吐，短時(shí)進(jìn)入危象 服鎮(zhèn)靜或安息藥可無上述表現(xiàn)服鎮(zhèn)靜或安息藥可無上述表現(xiàn)危象危象出現(xiàn)嚴(yán)重低血糖、昏迷、休克出現(xiàn)嚴(yán)重低血糖、昏迷、休克1、低血糖及低血糖昏迷、低血糖及低血糖昏迷 神志改動(dòng)、嗜睡、朦朧或焦躁、呻吟，面部或四肢肌肉抽動(dòng)，交感神志改動(dòng)、嗜睡、朦朧或焦躁、呻吟，面部或四肢肌肉抽動(dòng)，交感神經(jīng)興奮病癥，神經(jīng)興奮病癥， 既而

25、昏迷既而昏迷2、感染誘發(fā)昏迷、感染誘發(fā)昏迷 高熱、厭食、嘔吐、神志朦朧，血壓下降高熱、厭食、嘔吐、神志朦朧，血壓下降3、其他緣由引起昏迷、其他緣由引起昏迷 鎮(zhèn)靜、安息藥物鎮(zhèn)靜、安息藥物 水中毒：嘔吐、冷淡、嗜睡、癲癇發(fā)作水中毒：嘔吐、冷淡、嗜睡、癲癇發(fā)作 低體溫：冬季、黏液性水腫病人低體溫：冬季、黏液性水腫病人危象危象4、休克： 與上述有重疊 面色慘白、厭食、惡心、焦躁、反響愚鈍、脈率快、冷汗血壓下降為明顯目的、末梢紫紺等 緣由：多緣由 腎上腺皮質(zhì)功能低 失水 低血鈉 感染 低血糖5、精神病樣發(fā)作：發(fā)病快、無前期、多因勞累、未進(jìn)食或停頓治療后 焦躁不安、自言自語、幻聽、幻視、喊叫、狂躁 4小時(shí)

26、 昏迷三、誘因三、誘因 感染、勞累、停頓治療、服用鎮(zhèn)靜劑感染、勞累、停頓治療、服用鎮(zhèn)靜劑感染：感染： 占占70% 呼吸道感染呼吸道感染 消化道感染消化道感染 泌尿道感染泌尿道感染實(shí)驗(yàn)室檢查實(shí)驗(yàn)室檢查1、垂體前葉激素測(cè)定、垂體前葉激素測(cè)定 根底程度根底程度 病癥體征及單次血不能判別時(shí)行病癥體征及單次血不能判別時(shí)行 LHRH ,TRH,低血糖實(shí)驗(yàn)，以判低血糖實(shí)驗(yàn)，以判別別 垂體功能垂體功能 危象時(shí)不能等結(jié)果，抽血待測(cè)危象時(shí)不能等結(jié)果，抽血待測(cè)2、血糖、電解質(zhì)、腎功能、血糖、電解質(zhì)、腎功能 血糖可低至血糖可低至1.12mmol/L(20mg/dl) 50% 有低血鈉有低血鈉 少數(shù)有低血鉀少數(shù)有低血鉀

27、 50%以上以上BUN升高升高診斷及鑒別診斷診斷及鑒別診斷重點(diǎn)：重點(diǎn)： 病史、病癥、體征病史、病癥、體征 有生育史婦女如有昏迷、休克、精神樣發(fā)作、低血糖有生育史婦女如有昏迷、休克、精神樣發(fā)作、低血糖 思索垂體前葉功能低下思索垂體前葉功能低下 體檢毛發(fā)稀少、皮膚色素淡支持診斷體檢毛發(fā)稀少、皮膚色素淡支持診斷 鑒別診斷鑒別診斷 胰島細(xì)胞瘤：昏迷前無惡心、厭食胰島細(xì)胞瘤：昏迷前無惡心、厭食 肝?。河懈尾∈?、肝功異常肝?。河懈尾∈?、肝功異常 原發(fā)腎上腺皮質(zhì)功能低減：有典型皮膚色素冷靜原發(fā)腎上腺皮質(zhì)功能低減：有典型皮膚色素冷靜 Replacement therapy Hypopituitarism an

28、d panhypopituitarism are treated by replacement of appropriate hormones. Since most of the anterior pituitary hormones are proteins or glycoproteins released in pulsatile patterns, whose functions are to induce secretion of smaller molecule hormones (thyroid hormones and steroids), it is simpler and

29、 less expensive for most purposes to simply replace the target gland hormones. There are a few exceptions, such as fertility induction. GH is replaced with growth hormone. TSH is replaced with thyroxine. ACTH is usually replaced with hydrocortisone but any glucocorticoid may be used. LH and FSH are

30、most often replaced by supplying the appropriate sex steroids (e.g., testosterone or estrogen and progestin). Virtually all people who need T or E2 replacement for hypopituitarism rarely have spontaneous, effective spermatogenesis or follicular maturation. Both GnRH by subcutaneous pump and gonadotropins (Pergonal) by daily subcutaneous injections have been used effectively to induce fertility. Prolactin is not usually replaced, as infant formula is readily available, simpler, and much cheaper. ADH is replaced most commonly with oral, nasal, and sometimes int