1、浙医二院内分泌科 任跃忠2010 12 112010 12 11.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践vThe sellar蝶鞍region is a site of various types of tumors.Pituitary adenomas are the most common and account for 10-15%of all intracranial tumors.vTumors exceeding 10 mm are defined as macroadenomas
2、,and those smaller than 10 mm are termed microadenomas.vMost pituitary adenomas are microadenomas.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践PathophysiologyPituitary macroadenomas are benign epithelial neoplasms composed of adenohypophysial cells.Primary malignant tumors o
3、f the pituitary are extremely rare.Pituitary tumor development is a monoclonal process with several contributing factors.arise from a mutated pituitary cell.remain unknown.Some pituitary tumors may occur as part of a clinical syndrome.In multiple endocrine neoplasia type 1(MEN 1).胆道疾病病人护理化工企业本质安全理论实
4、践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践FrequencyUnited StatesPituitary tumors are found on autopsy in as many as 25%of unselected cases.The annual incidence of pituitary neoplasms varies from 1-7 cases per 100,000 population based on neurosurgical series.Mortality/MorbidityMorbidity in p
5、ituitary macroadenomas varies from incidentally discovered nonfunctioning tumors to disabling macroadenomas.Morbidity results from mass effects(eg,bitemporal hemianopsia双颞侧偏盲),hormonal imbalance(pituitary hormone deficiency due to compression of the normal pituicytes or hormonal excess from the tumo
6、r),and patient comorbidities.Significant morbidity is also associated with treatment of these tumors.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践RaceNo racial predilection exists for pituitary macroadenomas.SexAutopsy series show an equal distribution of pituitary tumors be
7、tween men and women.Corticotropinomas.mainly in women,.4:1.Amenorrhea(or menstrual irregularities),which is a relatively common symptom in women with macroadenomas,raises the suspicion of a pituitary lesion.AgeTumors affect individuals of all ages,but incidence increases with age,peaking between the
8、 third and sixth decades of life.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践ClinicalPatients with pituitary macroadenomas may be asymptomatic or may present with complaints due to hormonal imbalance or mass effects.effects depend on the hormones involved.Panhypopituitarism
9、 spared.The larger the tumor,the more likely it is to involve most hormones.The most sensitive are the somatotrophs and the gonadotrophs,whereas corticotrophs and thyrotrophs tend to be more resistant.Hyperprolactinemia presents with hypogonadism,infertility不育,amenorrhea,and galactorrhea乳溢.Hyperprol
10、actinemia can be due to increased hormone production by a prolactinoma,or it can be the result of stalk compression by the macroadenoma regardless of hormone activity.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Corticotropin excess presents with Cushing disease.Corticotropi
11、nomas are rarely macroadenomas.Corticotropin suppression due to compression of the normal corticotrophs presents with glucocorticoid insufficiency.The clinical picture of secondary glucocorticoid deficiency is much milder than.Thyrotropin excess presents with secondary hyperthyroidism.Thyrotropinoma
12、s are very rare tumors.They present most frequently as macroadenomas.naturally aggressive.presents with secondary hypothyroidism.Excess growth hormone presents with acromegaly as the result of a somatotropinoma(often a macroadenoma),while inadequate growth hormone presents with failure to thrive in
13、children but often no complaints in adults.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Gonadotropinomas most often are asymptomatic and usually secrete inactive follicle-stimulating hormone(FSH)and luteinizing hormone(LH)-like glycoproteins and/or alpha subunit.They often a
14、re macroadenomas and usually result in hypopituitarism.Rarely,they can lead to testicular enlargement in men and ovarian hyperstimulation in women.Mass effects of the macroadenoma may present with visual deficits,headache,elevated intracranial pressure,or intracranial hemorrhage.胆道疾病病人护理化工企业本质安全理论实践
15、及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Pituitary apoplexy垂体卒中 as a medical emergency with a headache,sudden collapse暴脱,shock,and death if not treated emergently.This tends to occur in macroadenomas.Administration of stimulatory agents,such as thyroid-stimulating hormone TSH,gonadotropin-r
16、eleasing hormone(GnRH),and insulin-hypoglycemia,have been postulated to lead to increased metabolic needs by the macroadenoma(which has deficient blood supply),leading to necrosis.Nelson syndrome results from treatment of Cushing disease with bilateral adrenalectomy.The lack of negative glucocortico
17、id feedback is postulated to lead to excessive tumor growth.Such tumors are much more aggressive and locally invasive compared to the usual corticotroph adenomas.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Most patients do not have physical findings associated with macroade
18、nomas.Physical findings may be attributable to the mass effects or hormonal disruption.When the tumor extends onto the optic chiasm视交叉,visual field deficits may be demonstrable.Sudden increase in tumor size,such as can occur with hemorrhage,may lead to elevated intracranial pressure.Hormonally activ
19、e tumors might present with symptoms due to target organ stimulation,such as hyperthyroidism,Cushing syndrome,or hyperprolactinemia.CausesThe cause of pituitary macroadenomas is unknown.The most favored theory attributes monoclonal neoplastic transformation of pituitary cells as the cause of tumor i
20、nitiation and growth.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Differential DiagnosesTuberculosisOther Problems to Be Considered Lymphocytic hypophysitis淋巴细胞性垂体炎Pituitary hyperplasia(as in pregnancy,longstanding hypothyroidism,and ectopic hypothalamic factor production,su
21、ch as corticotropin-releasing hormone CRH or growth hormonereleasing hormone GHRH)Metastatic tumorsGerminomas生殖细胞瘤Granulomatous disease肉芽肿病(eg,tuberculosis,syphilitic gumma梅毒性树胶肿,sarcoidosis肉状瘤病)Vascular aneurysms动脉瘤Craniopharyngiomas颅咽管瘤Histiocytosis-x组织细胞增多病 Tumors of the clivus斜坡(eg,chordomas脊索瘤)
22、.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Workup-Workup-Laboratory StudiesLaboratory StudiesLaboratory tests include basal hormone levels and dynamic hormone measurements depending on the tumor studied.All tumors should have screening hormone measurements,which may inclu
23、de prolactin,thyrotropin,thyroxine,adrenocorticotropin,cortisol,LH,FSH,estradiol,testosterone,growth hormone,insulinlike growth factor-1(IGF-1),and alpha subunit glycoprotein.are performed to assess the functionality of a tumor and assist in differential diagnosis.They also can be used to assess ant
24、erior pituitary reserve.Thyrotropin-releasing hormone(TRH)causes elevation of serum prolactin and thyrotropin.Prolactinomas,hyperprolactinemic states,hyperthyroidism,and panhypopituitarism exhibit a blunted response.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践GHRH produces
25、an elevation in growth hormone.This response is blunted in growth hormone deficiency,Cushing disease,and hypothyroidism.Acromegaly may produce a paradoxical反常的reduction in growth hormone.Hyperglycemia suppresses serum growth hormone.This suppression does not occur in pituitary tumors secreting growt
26、h hormone,ectopic growth hormonereleasing tumors,Cushing syndrome,and anorexia厌食症nervosa.A paradoxical rise in growth hormone may be observed in acromegaly,acute illness,and chronic renal failure.CRH causes a rise in corticotropin.This response is exaggerated in Cushing disease but blunted in other
27、causes of Cushing syndrome.When combined with inferior petrosal sinus sampling岩下窦取血法,this test may assist in differentiating Cushing disease from benign ectopic adrenocorticotropic hormone(ACTH)syndrome.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Insulin-induced hypoglycemi
28、a causes a rise in corticotropin,cortisol,and growth hormone.A blunted response is observed in Cushing syndrome,growth hormone deficiency,hypothyroidism,and hyperthyroidism.Metyrapone美替拉酮causes a rise in morning serum 11-deoxycortisol and urinary 17-hydrocorticosteroids(17-OH steroids).An exaggerate
29、d response occurs in Cushing disease,but no response is observed in other causes of Cushing syndrome.Dexamethasone suppression testing is used in Cushing syndrome evaluation.An overnight 1-mg dexamethasone dose fails to suppress morning serum cortisol in Cushing syndrome but is only a screening test
30、.Low-dose and high-dose.Cosyntropin 1-24促肾上腺皮质激素testing and corticotropin infusion testing assist in assessing the hypothalamic-pituitary-adrenal axis for adrenocortical insufficiency.GnRH causes an increase in LH and FSH levels.This response is blunted in pituitary hypogonadism but exaggerated in p
31、rimary hypogonadism.Test results,however,are not very dependable.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Workup-Workup-Imaging StudiesPituitary imaging Plain skull radiographs are poor at delineating soft tissues.CT scanning is better at depicting bony structures and ca
32、lcifications within soft tissues than either plain radiography or MRI.such as germinomas生殖细胞瘤,craniopharyngiomas,and meningiomas脑(脊)膜瘤,are better determined with CT scanning MRI is more expensive than CT scans but is the preferred imaging study for the pituitary because it provides better visualizat
33、ion of soft tissues and vascular structures.No exposure to ionizing radiation occurs,T1-weighted images,fat produces highsignal intensity images.T2-weighted images of structures with high water content,such as cerebrospinal fluid and cystic lesions,produce high-intensity signals,while structures wit
34、h high fat content present with low-intensity signals.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Other TestsVisual field testing should be performed,especially in tumors involving the optic chiasm.The severity of visual defects may dictate a more aggressive treatment cours
35、e.Histologic FindingsThe histology of pituitary macroadenomas shows varying levels of neoplastic activity.Frozen sections are usually not dependable for definitive diagnosis.Hormonal immunohistochemical stains for neuroendocrine markers are useful,especially in the nonfunctioning tumors.胆道疾病病人护理化工企业
36、本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Treatment Medical Care The goal of treatment is complete cure.When this is not attainable,reducing tumor mass,restoring hormone function,and restoring normal vision are attempted using medications,surgery,and radiation.Pituitary macroadenomas
37、 often require surgical intervention for cure.The exceptions to this rule are the macroprolactinomas,.Prolactin-secreting macroadenomas respond to dopaminergic agonists.The most frequently employed medications include bromocriptine,cabergoline卡麦角林.Quinagolide喹高利特is an alternative with fewer adverse
38、effects than bromocriptine.(For prolactin-secreting microadenomas,surgical removal is followed by recurrence in about 50%of patients.Therefore,medical therapy is preferred.).胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Growth hormone-secreting tumors should be treated surgic
39、ally,often followed by radiation therapy.Radiation therapy results in 50%reduction in growth hormone levels within 2 years,followed by an additional 25%in the following 2 years.Medical treatment is used after surgery to suppress growth hormone secretion,awaiting the occurrence of the effects of radi
40、otherapy.Octreotide善得定is the treatment of choice.Growth hormone receptor antagonists have been another addition to the treatment of acromegaly.Dopamine agonists also may be used but are not as effective as octreotideCorticotropin-secreting pituitary tumors are treated using surgery and radiation the
41、rapy(however,they are rather radioresistant).Medical therapy is reserved for patients whose therapy fails,.Centrally acting medications.include bromocriptine,valproic acid丙戊酸,and cyproheptadine丙戊酸.Peripherally acting agents include ketoconazole,mitotane米托坦,and metyrapone美替拉酮.胆道疾病病人护理化工企业本质安全理论实践及方法内
42、科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Gonadotropin-secreting macroadenomas are treated surgically,followed by radiation.Bromocriptine or octreotide may be used.Nonsecretory macroadenomas are treated surgically.If surgery is contraindicated,octreotide or bromocriptine may be tried;however,the
43、 results are often disappointing.Thyrotropin-secreting tumors are treated surgically,followed by radiation therapy.Octreotide is quite effective in such tumors.Traditional radiotherapy using external beam radiation is used to complement surgery in inoperable cases or in patients declining surgery.pa
44、nhypopituitarism.Radiosurgery using a Gamma knife employs a computer-assisted stereotactic mapping followed by several discrete radiation treatment fields to the tumor.Incidence of hypopituitarism is less.However,long-term studies are not available yet.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经
45、验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践Treatment Surgical CarePituitary macroadenomas often require surgical extirpation for cure.Transsphenoidal经蝶骨的surgery is the approach of choice.Only about 1%a transcranial approach.Compared with remission rates of 90%in microadenomas,macroadenomas.15-37%when treated with surge
46、ry alone.Radiation therapy and medical treatment often complement surgery.ConsultationsWhen a pituitary macroadenoma is diagnosed,consultations with an endocrinologist,neurosurgeon,neuroradiologist,and neurophthalmologist神经眼科学 should be considered.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫
47、康复训练手册偏执性精神障碍品管圈实践以以GHGH瘤为例瘤为例中国肢端肥大症诊治规范:治疗目标是随机血清GH2.5ug/L;口服葡萄糖负荷血清GH水平1.0ug/L;血清IGF水平下降;肿瘤消除或缩小;临床症状消除或减轻;尽可能保留内分泌功能或进行激素替代。SSA治疗适应症:术前,缩小肿瘤体积;肿瘤切除后残余肿瘤的辅助治疗;放射治疗后的过渡治疗;并发症治疗。.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践GHGH瘤的治疗流程瘤的治疗流程:定性诊断-定位诊断-垂体功能的全面评估-并发症评估v 血压、血糖、
48、心肺功能评估:血压、血糖术前应达到安全水平v 所有垂体、靶腺内分泌功能评估:甲状腺功能纠正与皮质功能替代之间的关系v 术前使用缩小肿瘤的药物(溴隐停、生长抑素)尹剑 等。中华神经外科杂志 2006年 22卷 6期 343-344.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践:定期评估,长期随诊(推荐1年1次)v 纠正电解质紊乱v 垂体激素替代v 注意垂体周围组织损伤v 下丘脑综合征v 尿崩症v SIADHv 脑耗盐综合征.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病
49、常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践:血糖、血压、骨代谢、心功能、肾功能(专家小组包括:内分泌专家、神经外科专家、放射治疗专家、放射诊断学专家、病理学专家。).胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践v内分泌激素替代:最好的方法是将病人移交给内分泌专科 没有内分泌专科的医院应注意以下问题:靶腺激素替代:甲状腺素、皮质激素、性 激素、保持骨代谢正常v三个月随访v有应激情况要增加替代剂量.胆道疾病病人护理化工企业本质安全理论实践及方法内科护理学呼吸系统总论概论脾胃病常见症状
50、及治疗经验偏瘫截瘫康复训练手册偏执性精神障碍品管圈实践MedicationMedications are used to control excess hormone secretion or to replace deficient hormones.Dopaminergic agentsThese agents directly stimulate postsynaptic突触后的dopamine receptors.The dopaminergic neurons in the tuberoinfundibular结节漏斗部的process modulate the secretion