肢端肥大症的并发症

肢端肥大症可合并多种并发症,本文简要介绍肢端肥大症合并心血管系统疾病、糖代谢异常、骨关节病变等并发症,以及经手术、放疗等治疗后这些并发症的转归。     

肢端肥大症患病率的调查

肢端肥大症患病率的调查陆小平韩松崔岚萨仁图雅袁飞雁晏玲赵东肢端肥大症是成人垂体前叶生长激素分泌瘤分泌生长激素（ｈＧＨ）过多而引起的疾病。其在人群中的患病情况国内外报道较少，为了解本病的患病率，我们进行了专项调查，结果如下：一、对象和方法１．对象：为１...     

肢端肥大症性心脏病伴顽固性心力衰竭1例

患者 ,男 ,32岁。因反复出现劳力性呼吸困难半年余 ,加重 1周入院。患者于半年前反复出现心慌、胸闷、呼吸困难等症状 ,多以劳累后诱发 ,再次入我科治疗。患者始于 6年前出现手足肥大 ,相貌改变 ,性欲亢进 ,易发怒 ,经脑ＣＴ检查示蝶鞍增大 ,符合垂体瘤征象 ,血浆生长激素 (ＧＨ) 1 34μｇ/Ｌ ,未给予特殊治疗。至 2 0 0 0年 7月在某医院行伽玛刀垂体瘤切除术 ,术后 3个月反复出现心力衰竭、血压增高等 ,两次住院均给予以强心、利尿、扩血管等综合治疗 ,心力衰竭基本纠正 ,血压正常 ,自动出院。本次入院血压 1 5 0 /1 0 0ｍｍＨｇ( 1ｍｍＨ…     

以糖尿病为首发表现的肢端肥大症两例

病例1:患者,男性,59岁.因"口干、多饮、多食伴消瘦半个月余"入住我科.测血糖18 mmol/L,考虑初发糖尿病.家族无类似病史.体格检查:体温37℃,呼吸20次/分,脉搏80次/分,血压110/70 mm Hg(1 mm Hg =0.133 kPa),体重45 kg,身高172 cm,体质量指数23.83 kg/m2,肢端肥大面容,体型中等,余心、肺、腹无异常.实验室检查:空腹血糖(FBG) 12.4mmol/L,餐后2小时血糖(2hBG) 19.3 mmol/L,空腹C肽0.48ng/ml(0.81 ～ 3.85ng/ml),餐后2小时C肽0.52 ng/ml,糖化血红蛋白比例(HbA1C) 12.42％,抗胰岛素抗体、抗胰岛细胞抗体、抗谷氨酸脱羧酶抗体均阴性,高糖抑制试验:生长激素(GH)(0'):25.9 μg/l,生长激素(30'):15.4 μg/l,生长激素(60'):18.3 μg/L,生长激素(120'):30.7 μg/L,类胰岛素样生长因子-1(IGF-1)555 μg/l,肝肾功能电解质正常,垂体前叶其他激素(甲状腺功能、促肾上腺皮质激素、皮质醇、睾酮、黄体生成素及卵泡生成素)正常.     

肢端肥大症性心肌病2例

例 1　女 ,43岁。发现颜面发育异常 1 0余年 ,反复胸闷、气短伴双下肢水肿 1年 ,加重 3d入院。1年前发现“垂体瘤”,未治疗。 3年前闭经。入院时体检 :血压 ( BP) 1 2 0 /70 mm Hg( 1 mm Hg=0 .1 33k Pa) ,端坐呼吸 ,皮肤粗糙 ,多汗、多毛。前额骨、眉弓、颧骨弓及鼻增大、突出 ,左眼失明 ,口唇增厚 ,舌大而厚 ,语音低顿。双侧颈静脉怒张 ,双肺底细小水泡音 ,心尖搏动于 肋间左锁骨中线外 3.0 cm,心率 1 0 2次 /min,律不齐 ,心尖部 级收缩期吹风样杂音。肝大肋下 4.0 cm,双下肢重度水肿。超声心动图示左室 ( LV) 70 mm,左房 ( LA) 55m…     

肢端肥大症伴甲状腺多发结节一例

患者,女, 41岁,因“面部改变伴手指、足趾增粗 4年”入院。入院体检:Bp120 /80mmHg,颧骨、眉弓、下颌突出,鼻大,口、唇、舌体肥厚,手足粗大、皮肤增厚粗糙,手臂、背、臀、面部痤疮多发,语音低沉,无视野缺损。甲状腺未触及肿大,心肺体检无殊。辅助检查:血清基础生长激素(GH) >40μg/L,明显高于正常。葡萄糖抑制试验血浆GH不能被抑制。甲状腺功能全套:总甲状腺素(TT4 ) 55. 5nmol/L(正常范围 58. 0 ~161. 0nmol/L),游离三碘甲腺原氨酸 (FT3 ) 2. 70pmol/L(正常范围3. 23 ~7. 22pmol/L)、游离四碘甲腺原氨酸(FT4 )8. 90pmol/L(正常…     

肢端肥大症11例并发症临床分析

目的研究肢端肥大症并发症发生情况。方法选取浙江大学附属第一医院2000~2004年收治的11例肢端肥大症患者进行回顾性分析。结果肢端肥大症并发症发生率分别为:糖尿病72.7%、脂代谢紊乱45.5%、心血管疾病45.5%、甲状腺疾病18.2%。结论有效地控制肢端肥大症原发病可减少其并发症的发生或延缓其并发症的发展。     

OVERNIGHT URINARY GROWTH HORMONE MEASUREMENT IN THE DIAGNOSIS OF ACROMEGALY

Several studies report higher urinary GH excretion in acromegalic patients compared to control subjects. We investigated the diagnostic potential of overnight urinary GH excretion in acromegaly, using a recently developed enzyme-linked immunosorbent assay. Overnight urine samples were obtained from 117 control subjects and nine patients with untreated acromegaly. GH excretion was higher in acromegalic patients compared to control subjects, with geometric mean total overnight values of 46.35 and 5.73 microU respectively. The range for total overnight urinary GH in control subjects was 0.75-21.75 microU and two of the nine patients with untreated acromegaly had GH measurements within this range. Urinary GH measurements were corrected using predictive clinical variables but this resulted in minimal improvement in discrimination between control subjects and acromegalic patients. Lack of complete discrimination between control subjects and acromegalic patients limits the usefulness of a single overnight urinary GH measurement as a screening test for acromegaly.     

肢端肥大症患者血清生长激素和生长介素的相互关系及在评定病情活动性中的价值

作者研究了２３０例肢端肥大症患者的血清ＩＲ－ｈＧＨ和ＳＭＣ３项指标的相互关系及在判断病情活动性中的价值。观察到ＯＧＴＴ时血清ＴＲ－ｈＧＨ的空腹值和谷值与血清ＳＭＣ浓度作对数转换后有很明显的正相关（ｒ＝０．５０３～０．５６３８，Ｐ均＜０．００５）。４０３例次空腹血清ＩＲ－ｈＧＨ与ＳＭＣ之间呈一曲线关系，只有消退ＩＲ－ｈＧＨ＜２０μｇ／Ｌ时，二者方呈直线关系。ＴＲ－ｈＧＨ在２０～１５０μｇ／Ｌ之间．ＳＭＣ的浓地度是平的。在３组病人分析了血清ＩＲ－ｈＧＨ和ＲＲ－ｈＧＨ在ＯＧＴＴ的谷值及血清ＳＭＣ浓度与病情活动性的平均符合率分别为９１．７％、９４．７％和９１．７％。以上结果说明肢端肥大症患者血清ＴＲ－ｈＧＨ、ＲＲ－ｈＧＨ与ＳＭＣ的相互关系密切，但ＴＲ－ｈＧＨ与ＳＭＣ之间并不呈直线关系。３项指标均能较好反映临床病情的活动性，但以ＲＲ－ｈＧＨ最佳。     

BROMOCRIPTINE SUPPRESSION OF PLASMA GROWTH HORMONE IN ACROMEGALY

Twelve acromegalic patients were studied in one or more of three protocols to establish the response of plasma growth hormone (hGH) concentraions to (a) a single oral test dose of bromocriptine, (b) incremental dose therapy from 10 mg/day to 40 mg/day over 4 weeks, and (c) sustained therapy with 20 mg/day over a 3 month period. Ten of the patients studied had previously been treated by yttrium implantation, external pituitary irradiation or surgical hypophysectomy. A high incidence of side effects including postural hypotension, hallucinations and peripheral vasospasm was noted reducing the numbers of patients completing the three protocols. Suppression of hGH concentrations was disappointing; significant reductions in hGH concentraions occurred in five of eleven patients in response to a single test dose of bromocriptine, and in only one of seven patients treated with the drug over a sustained period. An impression of clinical improvement was gained in one patient, but there was no associated reduction in plasma hGH concentrations. It seems likely that acromegalic patients who have failed to respond to conventional treatment may be more resistant to bromocriptine therapy.     

RESOLUTION OF ACROMEGALY AFTER REMOVAL OF A BRONCHIAL CARCINOID SHOWN TO SECRETE GROWTH HORMONE RELEASING FACTOR

A 29 year old woman with an enlarged pituitary fossa and classical acromegaly, possibly present for ten years, had biochemical and partial somatic resolution of the disorder after removal of a bronchial carcinoid tumour. In addition, galactorrhea stopped, menstruation returned after two years, and amenorrhea and elevated prolactin levels fell towards normal.
Immunocytochemistry showed numerous growth hormone releasing factor (GRF) staining cells in the tumour. The tumour cells, when cultured, produced a supernatant selectivity stimulating human pituitary somatotrophic cell cultures to produce growth hormone (GH). The bronchial carcinoid did not secrete detectable GH, but extracts of it, and preoperative serum contained GRF immunoreactivity which coeluted with synthetic human pancreatic GRF.     

THE EFFECT OF CALCITONIN ON GROWTH HORMONE SECRETION IN ACROMEGALY

To determine whether human calcitonin inhibits GH secretion in acromegaly, as previously described for healthy subjects, the effect of an i.v. bolus injection of calcitonin or saline on GH levels in patients with active acromegaly was studied and compared to that of an i.v. bolus injection of the synthetic somatostatin analogue, octreotide. After the injection of calcitonin, GH levels decreased by 46% of initial values, whereas octreotide reduced GH levels by 87% and saline had no significant effect. Administration of calcitonin to acromegalics did not cause the transient rise in plasma PRL and TSH levels seen in normal subjects. Octreotide induced a decrease in plasma PRL in three out of seven patients. It is concluded that human calcitonin suppresses GH secretion in acromegaly, but not to normal levels; moreover the effect is less than that found for octreotide. In addition, acromegalic patients did not exhibit the PRL and TSH-releasing activity of calcitonin found in normal subjects, while octreotide inhibited PRL secretion in some acromegalic patients.     

Sandostatin 治疗肢端肥大症的疗效

本文报道用生长抑素八肽类似物(Sandostatin, SMS201—995)治疗10例活动性肢端肥大症20.3±7.5个月的结果。治疗剂量为300～1500μg/d,分3次,每8小时皮下注射1次。结合临床及血清GH及SMC值评定,在用药期间,2例控制正常,4例显效,3例有效,1例无效。5例新诊断患者用药6个月后1例的微腺瘤消失,4例大腺瘤高度由12.4±4.9mm降至9.8±4.4mm。8例患者有短暂的胃肠道反应,8例胆囊收缩功能减弱,4例糖耐量变坏。指出SMS201-995对肢端肥大症有疗效,但其治疗价值尚待进一步研究。     

FAILURE OF LONG-TERM CYPROHEPTADINE THERAPY IN LOWERING GROWTH HORMONE LEVELS IN ACROMEGALY

Basal and post-glucose growth hormone (GH) responses were evaluated in 10 acromegalics receiving cyproheptadine (cypro) 4 mg 6 hourly at 2 and 21 days after initiating therapy. Of the 10 patients, six had macroadenomas with varying degree of suprasellar extensions, one a microadenoma and three had persistent hypersomatotropism despite pituitary adenomectomy due to residual tumour. The basal and post-glucose GH showed no significant change in all, except one with a pituitary microadenoma. His GH was reduced from 680 to 108 mU/L on the second and 82 mU/I on the 21st day of cypro therapy. These data do not suggest any therapeutic role of cypro in the management of acromegaly.     

单层培养的垂体生长激素分泌瘤细胞对生长激素释放激素和生长抑素的反应

本文研究7例肢端肥大症患者垂体单纯生长激素(GH)分泌瘤单层培养细胞对GH释放激素(GRF)和生长抑素(SS)的反应。培养液中10~(-8)M GRF使5例瘤细胞GH分泌增加171.6±22.6％,10~(-7) M GRF使全部7例瘤细胞GH分泌明显升高到220.6±47.7％。10~(-9)～10~(-7) M SS使5例瘤细胞GH基础分泌下降到50.4±9.8％。同时加入10~(-8)或10~(-7) M的GRF和SS,在7例瘤细胞上均看到GRF兴奋GH分泌的作用被完全阻断。3例病人术前接受GRF和SS试验,血清GH水平的变化分别为对照的222.0±30.6％和10.7±4.7％。结果表明单层培养的垂体GH瘤细胞对GRF和SS仍有反应。     

THE INFLUENCE OF OCTREOTIDE TREATMENT ON PULSATILE GROWTH HORMONE RELEASE IN ACROMEGALY

The pulsatile release of GH was investigated in eight active acromegalic patients before and during a subcutaneous infusion of 300 micrograms octreotide/24 h for 4 weeks. The number of GH pulses increased from a basal value of 14.4/24h to 16.3/24h during octreotide therapy. At the same time the mean GH concentration, valley concentration, peak height and amplitude decreased significantly. The inhibitory effect of octreotide on pulse characteristics did not depend on the time of day. IGF-I levels also decreased significantly; in five patients normal levels were reached. IGF-I levels correlated significantly with the mean GH level (r = 0.714, P less than 0.001), mean valley concentration (r = 0.697, P less than 0.001) and, to a lesser extent, area under the curve (r = 0.436, P = 0.033), but not the number of pulses. Plasma octreotide levels did not correlate with pulse parameters. In all but one patient a circadian rhythm was present during both the basal study and octreotide therapy. Compared with surgically treated acromegalics, the number of GH pulses was higher in untreated and octreotide-treated patients. This study demonstrates the pulsatile release of GH in active acromegaly both before and during octreotide therapy. This result suggests that endogenous GHRH is important for the generation of GH pulses in this disease.     

GROWTH HORMONE MODULATION OF ARGININE-INDUCED GLUCAGON RELEASE: STUDIES OF ISOLATED GROWTH HORMONE DEFICIENCY AND ACROMEGALY

Plasma glucagon and insulin responses to l -arginine were compared in normal controls and patients with isolated growth hormone deficiency and acromegaly. Patients with isolated growth hormone deficiency were characterized by high plasma glucagon response and low plasma insulin response, whereas acromegalic patients showed exaggerated plasma glucagon response and almost normal insulin response. These results suggest that growth hormone is probably required for optimum function of the islets, and since hyperglucagonaemia was observed in both growth hormone deficiency and acromegaly, metabolic disturbances stemming from the respective primary diseases may affect glucagon secretion.