1、垂体前叶功能减退垂体前叶功能减退解放军总医院内分泌科窦京涛垂体解剖 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 TSH(thyroid-stimulati
2、ng 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:ADH(antidiuretic hormone)-to increa
3、se 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 definition used by endocrinologists and is
4、 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 hypopituitarismDeficient pituitary gland function can result from damage to either the pituitary or the area just above the pituitary,the
5、 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.Injury to the pituitary can occur from a variety of
6、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 predictable loss of hormonal function with increasing
7、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 release)and finally the last to be lost is typically ACTH(whic
8、h controls adrenal function).Sheehans SyndromeSheehans 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 and leads to hypopituitarism following the birth.Deficiency of ACT
9、H and cortisolDeficiency 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 may include weight loss,fatigue,weakness,depression,apathy,nausea,vomitin
10、g,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 serum potassium),and vascular collapse(low blood pressure and shock)which can be fatal.
11、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 steroids is essential to reverse the crisis.Deficiency of TSH and thyroid hormoneDeficiency of thyr
12、oid 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.This constellation of symptoms is very uncomfortable and is often the symptom complex that drives patien
13、ts 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.Deficiency of LH and FSH(Hypogonadotropic Hypogonadism)Women develop ovarian suppression with irregular pe
14、riods or absence of periods(amenorrhea),infertility,decreased libido,decreased vaginal secretions,breast atrophy,and osteoporosis.Blood levels of estradiol are low.Estrogen should be replaced and can be given orally as Premarin or estrace,or can be given as a patch applied twice weekly.Women taking
15、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 decreased libido,impotence,decreased ejaculate volume,loss of body and facial hair,weakness,fatigue and often anemia.O
16、n 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 a gel preparation.In other countries,oral preparations of testosterone are available.Growth Hormone DeficiencyGrowth hormone is
17、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 maintaining an adequate energy level,optimal cardiovascular status and some mental functions.Symptoms of GH deficiency in adults include fatigue,
18、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 deficiency causing diabetes insipidusThis problem arises from damage to the pituitary stalk or the posterior pituitary gland.It may occur trans
19、iently after transsphenoidal surgery but is rarely permanent.Patients with diabetes insipidus have increased thirst and urination.Replacement of antidiuretic hormone resolves these symptoms.Antidiuretic hormone(ADH)is currently replaced by administration of DDAVP(also called Desmopressin)a synthetic
20、 type of ADH.DDAVP can be given by subcutaneous injection,intranasal spray,or by tablet,usually once or twice a day.主要原因主要原因一、原发一、原发、垂体缺血性坏死：产后大出血（、垂体缺血性坏死：产后大出血（Sheehan)综合征、糖尿病、颞综合征、糖尿病、颞动脉炎、子癫等动脉炎、子癫等、垂体区肿瘤：原发于鞍内的肿瘤，如嫌色细胞瘤、颅咽管瘤；、垂体区肿瘤：原发于鞍内的肿瘤，如嫌色细胞瘤、颅咽管瘤；鞍旁肿瘤：脑膜瘤、视神经胶质瘤。鞍旁肿瘤：脑膜瘤、视神经胶质瘤。、垂体卒中：一般与垂
21、体瘤有关、垂体卒中：一般与垂体瘤有关、医源性鼻烟部或蝶鞍区放射治疗后、手术创伤毁坏、医源性鼻烟部或蝶鞍区放射治疗后、手术创伤毁坏、免疫性疾病、免疫性疾病、感染性疾病、感染性疾病、海绵窦血栓形成及原发性空泡蝶鞍、海绵窦血栓形成及原发性空泡蝶鞍主要原因主要原因继发性继发性、垂体柄破坏性外伤、肿瘤或动脉瘤压迫及手术创伤、垂体柄破坏性外伤、肿瘤或动脉瘤压迫及手术创伤、下丘脑或其他中枢神经系统病变创伤、恶性肿瘤、下丘脑或其他中枢神经系统病变创伤、恶性肿瘤、类肉瘤、异位松果体瘤及神经性厌食等类肉瘤、异位松果体瘤及神经性厌食等机理机理 缺血缺血 破坏破坏临床表现临床表现一、垂体前叶机能减退一、垂体前叶机能减
22、退1、PRL和和GH分泌不足：分泌不足：PRL和和GH分泌不足是产后大出血最先出现分泌不足是产后大出血最先出现 的垂体激素分泌受损表现的垂体激素分泌受损表现 PRL 产后无乳、乳房不胀产后无乳、乳房不胀 GH 血糖血糖 2、促性腺激素不足：产后闭经、性欲减退、阴毛脱落、乳房萎缩及内、促性腺激素不足：产后闭经、性欲减退、阴毛脱落、乳房萎缩及内 外生殖器萎缩和不育外生殖器萎缩和不育 临床表现临床表现促甲状腺激素分泌不足促甲状腺激素分泌不足:表情淡漠、反应迟钝、怕冷、健表情淡漠、反应迟钝、怕冷、健 忘、面色苍白、眉毛头发稀少、忘、面色苍白、眉毛头发稀少、心率慢、可有或无黏液性水肿心率慢、可有或无黏液
23、性水肿促肾上腺皮质激素分泌不足：无力、食欲不振、不耐饥促肾上腺皮质激素分泌不足：无力、食欲不振、不耐饥 饿、体重减轻、心界缩小、心音饿、体重减轻、心界缩小、心音 低、血压低、抵抗力差低、血压低、抵抗力差危象危象危象前期：危象前期：某些诱因作用某些诱因作用 精神、神志症状：精神、神志症状：严重软弱无力、精神萎靡、表情淡漠、嗜睡、严重软弱无力、精神萎靡、表情淡漠、嗜睡、消化系统症状：消化系统症状：厌食、恶心、呕吐，进食或饮水既吐，合并中上腹痛，持续厌食、恶心、呕吐，进食或饮水既吐，合并中上腹痛，持续2-4周周 消瘦、脱水消瘦、脱水 心血管系统：心血管系统：收缩压收缩压80-90mmHg，脉压差小，
24、体位性低血压，脉压差小，体位性低血压 体温正常或高热体温正常或高热 高热伴恶心，呕吐，短时进入危象高热伴恶心，呕吐，短时进入危象 服镇静或安眠药可无上述表现服镇静或安眠药可无上述表现危象危象出现严重低血糖、昏迷、休克出现严重低血糖、昏迷、休克1、低血糖及低血糖昏迷、低血糖及低血糖昏迷 神志改变、嗜睡、朦胧或烦躁、呻吟，面部或四肢肌肉抽动，交感神志改变、嗜睡、朦胧或烦躁、呻吟，面部或四肢肌肉抽动，交感神经兴奋症状，神经兴奋症状，既而昏迷既而昏迷2、感染诱发昏迷、感染诱发昏迷 高热、厌食、呕吐、神志朦胧，血压下降高热、厌食、呕吐、神志朦胧，血压下降3、其他原因引起昏迷、其他原因引起昏迷 镇静、安眠
25、药物镇静、安眠药物 水中毒：呕吐、淡漠、嗜睡、癫痫发作水中毒：呕吐、淡漠、嗜睡、癫痫发作 低体温：冬季、黏液性水肿病人低体温：冬季、黏液性水肿病人危象危象4、休克：休克：与上述有重叠与上述有重叠 面色苍白、厌食、恶心、烦躁、反应迟钝、脉率快、冷汗面色苍白、厌食、恶心、烦躁、反应迟钝、脉率快、冷汗血压下降为明显指标、末梢紫绀等血压下降为明显指标、末梢紫绀等 原因：多原因原因：多原因 肾上腺皮质功能低肾上腺皮质功能低 失水失水 低血钠低血钠 感染感染 低血糖低血糖5、精神病样发作：发病快、无前期、多因劳累、未进食或停止治疗、精神病样发作：发病快、无前期、多因劳累、未进食或停止治疗后后 烦躁不安、自
26、言自语、幻听、幻视、喊叫、狂躁烦躁不安、自言自语、幻听、幻视、喊叫、狂躁 4小时小时 昏迷昏迷三、诱因三、诱因 感染、劳累、停止治疗、服用镇静剂感染、劳累、停止治疗、服用镇静剂感染：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实验室检查实验室检查1、垂体前叶激素测定、垂体前叶激素测定 基础水平基础水平 症状体征及单次血不能判断时行症状体征及单次血不能判断时行 LHRH,TRH,低血糖试验，以判低血糖试验，以判断断 垂体功能垂体功能 危象时不能等结果，抽血待测危象时不能等结果，抽血待测2、血糖、电解质、肾功能、血糖、电解质、肾功能 血糖可低至血糖可低至1.12
27、mmol/L(20mg/dl)50%有低血钠有低血钠 少数有低血钾少数有低血钾 50%以上以上BUN升高升高诊断及鉴别诊断诊断及鉴别诊断重点：重点：病史、症状、体征病史、症状、体征 有生育史妇女如有昏迷、休克、精神样发作、低血糖有生育史妇女如有昏迷、休克、精神样发作、低血糖 考虑垂体前叶功能低下考虑垂体前叶功能低下 体检毛发稀少、皮肤色素淡支持诊断体检毛发稀少、皮肤色素淡支持诊断 鉴别诊断鉴别诊断 胰岛细胞瘤：昏迷前无恶心、厌食胰岛细胞瘤：昏迷前无恶心、厌食 肝病：有肝病史、肝功异常肝病：有肝病史、肝功异常 原发肾上腺皮质功能低减：有典型皮肤色素沉着原发肾上腺皮质功能低减：有典型皮肤色素沉着
28、Replacement therapyHypopituitarism and 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 hormone
29、s and steroids),it is simpler and 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
30、may be used.LH and FSH are 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 subcutan
31、eous 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 intravenous or subcutaneo
32、us desmopressin.Oxytocin is most important during labor and delivery at the end of pregnancy,and can be replaced in that circumstance by pitressin治疗治疗 针对病因治疗针对病因治疗 如如:肿瘤切除、抗感染等肿瘤切除、抗感染等1、营养及护理：营养及护理：高蛋白、高维生素，适量钠、钾、氯离子等高蛋白、高维生素，适量钠、钾、氯离子等 避免过度饮水避免过度饮水 避免疲劳、饥饿、寒冷、感染避免疲劳、饥饿、寒冷、感染2、内分泌激素替代、内分泌激素替代 1）糖皮质
33、激素）糖皮质激素 可的松可的松 25mg-37.5mg/日日 或氢化可的松首选或氢化可的松首选 20mg-30mg/日日 强的松强的松 5mg-7.5mg/日日 应激时增量应激时增量2-3倍倍 2)甲状腺激素甲状腺激素 L-甲状腺素甲状腺素 25 g/日日 始始 每每2周增加周增加25 g 至至75-100 g/日日治疗治疗性激素或促性腺激素性激素或促性腺激素 女性女性:人工周期人工周期:乙烯雌酚乙烯雌酚 0.5-1mg/日日 或乙炔雌二醇或乙炔雌二醇 0.02-0.05mg/日日 共共 25天天 安宫黄体酮安宫黄体酮 6-12 mg/日日 或黄体酮或黄体酮 10 mg/日日 肌肉注射肌肉注射
34、 共共 5天天 雄激素雄激素:丙酸睾丸酮丙酸睾丸酮 12.5-25 mg 1-2次次/周周 庚酸睾酮庚酸睾酮 50 mg/2月月 诱发排卵诱发排卵:HMG 75IU/2天天-至卵泡成熟至卵泡成熟-E2 增高增高 HCG500-1000/天天 2-3天天 促排卵促排卵治疗治疗男性男性 丙酸睾酮丙酸睾酮 25-50mg 2次次/周周 庚酸睾酮庚酸睾酮 200mg 1次次/2周周 HCG+HMG生长激素生长激素:应用后症状改善应用后症状改善,代谢正常代谢正常危象处理危象处理1.补糖补糖 50%葡萄糖葡萄糖40-60ml 10%葡萄糖液续葡萄糖液续 2.静滴氢化可的松静滴氢化可的松 200-400mg/日日 3.抗生素抗生素 4.纠正电解质紊乱纠正电解质紊乱:低钠低钠 5.低体温昏迷低体温昏迷:保温保温 T3 静滴静滴12.5g/6-12g/6-12小小时时 或甲或甲状状腺素腺素50g 50g 鼻鼻饲饲 注意注意:一定在一定在补补充糖皮充糖皮质质激素前提下激素前提下 6.6.水中毒水中毒:强的松强的松1010mg 或可的松或可的松50mg 或静注氢化可的松或静注氢化可的松25mg+25%葡萄糖葡萄糖40ml
