肾上腺大结节样增生一例报道

库欣综合征(CS)中15% ～20%是由肾上腺原因引起的,其中约10%的患者具有双侧肾上腺增生.肾上腺性CS中皮质醇分泌有时可受肾上腺异常膜受体(包括抑胃肽受体、精氨酸加压素受体、儿茶酚胺类受体、LH/hCG受体和5-羟色胺受体等)的调节.该文报道了1例促肾上腺皮质激素非依赖性大结节样肾上腺增生引起的CS患者,并通过体内试验证实了肾上腺异常膜受体的存在.     

肾上腺大结节样增生一例报道

库欣综合征（CS）中15%～20%是由肾上腺原因引起的,其中约10%的患者具有双侧肾上腺增生。肾上腺性CS中皮质醇分泌有时可受肾上腺异常膜受体（包括抑胃肽受体、精氨酸加压素受体、儿茶酚胺类受体、LH/hCG受体和5-羟色胺受体等）的调节。该文报道了1例促肾上腺皮质激素非依赖性大结节样肾上腺增生引起的CS患者,并通过体内试验证实了肾上腺异常膜受体的存在。     

Adrenal autotransplantation with A-V anastomosis for treatment of Cushing''s disease. A follow-up study of 31 cases.

Thirty-one patients with Cushing's disease were treated with bilateral adrenalectomy and partial left adrenal autotransplantation with A-V anastomosis. They were followed up for 1 to 5 years. Three patients failed after adrenal autotransplantation to the abdominal muscle with the left gland's central vein anastomosed to the inferior epigastric artery. Twenty-eight patients underwent adrenal autotransplantation to the omentum with the gland's vein anastomosed to the gastroepiploic artery. Steroid could be omitted a short period after operation and life-long replacement therapy was avoidable in most patients. A few patients subsequently developed hypocorticalism. Two died and one had recurrence of Cushing's disease. Clinical and animal experimental results showed that the omentum is suitable for adrenal autotransplantation with A-V anastomosis. Transabdominal bilateral adrenalectomy and adrenal autotransplantation to the omentum with A-V anastomosis are acceptable in the treatment of Cushing's disease. The subsequent development of hypocorticalism and recurrence of Cushing's syndrome are also discussed.

     

Bilateral adrenal myelolipomas with Cushing's syndrome

A 24-year-old woman with non-pituitary dependent Cushing's syndrome was found to have bilateral adrenal myelolipomas at surgery. These benign tumors consist of bone marrow and fat and are uncommon incidental findings that are discovered by computed tomography. Myelolipomas have rarely been associated with Cushing's syndrome, but the cause of the syndrome has not always been defined, because the reports preceded modern diagnostic methods. Careful examination of the adrenal glands from our patient showed a fusion of myelolipoma elements and adrenal cells without distinct adenomas or the typical nodular pattern of adrenal hyperplasia. This report suggests that adrenal myelolipomas and atypical hyperplasia of the zona fasciculata may be anatomically and functionally related.     

Value of computed tomography of the abdomen and chest in investigation of Cushing's syndrome

Computed tomography (CT) scans were performed on 37 patients with biochemically proved Cushing's syndrome to evaluate the role of CT in the investigation of this condition. CT rapidly and correctly identified all 15 adrenocortical tumours, distinguishing five carcinomas from the 10 adenomas. In ACTH-dependent Cushing's syndrome appreciable bilateral adrenal enlargement was common in patients with an ectopic source (6 of 10 cases), while those with a pituitary source usually had normal sized adrenals (9 of 10). Two patients with a history of over seven years had bilateral adrenal nodules. CT was more accurate in locating a primary ectopic source of ACTH (5 of 12 cases) than any other technique and was particularly valuable in detecting small (less than 1.5 cm) peripheral lung carcinoid tumours which may be undetectable by conventional x-ray techniques. Its speed, accuracy, and simplicity make CT the technique of choice both to show the adrenal anatomy and to locate a suspected ectopic ACTH-secreting tumour in patients with proved Cushing's syndrome.     

Adrenal histoplasmosis in Cushing's syndrome with bilateral adrenocortical nodular hyperplasia

Adrenal histoplasmosis in Cushing's syndrome of adrenal origin is rare. A patient with Cushing's disease with bilateral nodular hyperplasia and histoplasmosis of both the adrenal glands is described. The diagnosis of histoplasmosis was only made post operatively as the constitutional manifestations, besides being partially masked by hypercortisolism also resemble those of tuberculosis.     

A clinico-pathological study of Cushing's syndrome at the University Hospital of the West Indies and a review of the literature

Cushing's syndrome is an uncommon but important disease. Twenty-one confirmed cases of spontaneous Cushing's syndrome were documented at the University Hospital of the West Indies over a 21-year period. They were predominantly young females (F:M ratio of 17:4; mean age 25 years and 3 months). The commonest presenting symptoms were amenorrhoea (41%) and obesity (19%). Common clinical features were cushingnoid features (95%), hypertension (76%) and hirsutism (82%). Twenty-nine per cent had frank hyperglycaemia. Cushing's syndrome was due to Cushing's disease in 10 cases, adrenal adenoma in 3 and adrenal carcinoma in 2 cases. In 4 cases with presumed adrenal hyperplasia, the histology was either unavailable or was not consistent with the diagnosis. Two cases appear now to have had the ectopic ACTH Syndrome. Adrenalectomy was the commonest treatment offered. There were no intra-operative or post-operative deaths but recurrence was common after subtotal adrenalectomy in Cushing's disease. Twenty-seven per cent of the patients developed Nelson's syndrome, which was fatal in 50%. Long-term hormone replacement therapy was unnecessary after surgery for adrenal adenomas. Treatment of Cushing's syndrome was well tolerated by the patients.     

Surgical treatment of endocrine hypertension experience in India

Between 1980 and 1994, 162 cases of endocrine based hypertension were diagnosed and treated surgically. Seventy-nine cases (48.7%) of phaeochromocytoma, 63 cases (38.8%) of Cushing's syndrome, and 20 cases (12.3%) of Conn's syndrome were diagnosed. In phaeochromocytoma 75% of the tumours arose from the adrenal glands and 25% arose from the extra-adrenal sites. Cushing's syndrome was caused by adenoma (45%), diffuse bilateral adrenal hyperplasia (36%), pigmented macronodular hyperplasia (9%), and adrenal carcinoma (10%). The most common cause of Conn's syndrome was adenoma (95%) which arose mainly from the left adrenal gland (60%). In the present series the success rate of surgical treatment was 100% for phaeochromocytoma, 90% for Cushing's syndrome and 96% for Conn's syndrome. Trucut biopsy of the kidneys of these patients showed hypertensive changes, the moderate hypertension could be due to renal damage.     

双侧肾上腺全切肾上腺皮质细胞自体移植治疗柯兴氏病

本文报道采用双侧肾上腺切除、肾上腺皮质细胞移植治疗柯兴氏病的新方法。4例病人接受此法治疗,其中3例治疗后随访一年以上。1例不用任何激素替代而完全治愈。另2例仅用少量激素,文中介绍了肾上腺皮质细胞制备及移植方法。     

External irradiation of the hypophysis for Cushing''s disease

During the past 12 years 17 patients with Cushing's disease (bilateral adrenal hyperplasia secondary to excessive pituitary adrenocorticotrophic hormone) have been treated initially with external pituitary irradiation. Of the 15 patients who have had adequate follow-up, nine showed complete biochemical remission, and one showed biochemical improvement. There were no complications. It is therefore recommended that the first mode of therapy for all patients with Cushing's disease should be pituitary irradiation if the patient's clinical condition permits.     

ADRENAL INCIDENTALOMAS:ANALYSIS OF 126 CASES

Adrenalmassesdiscoveredbyimagingtechniquesforreasonsunrelatedtoadrenaldiseasesarecalleda-drenalincidentalomas(1-3).Incidentalomaisadefi-nition,notadiagnosis.Thedetectionofmoreinci-dentalomasisexpectedastheuseofabdominalimag-ingincreases,suchasultrasound,computedtomog-raphy(CT)andmagneticresonanceimaging(MRI).Thecurrentprevalenceofunsuspectedadrenalmassesisapproximately1%to5%inabdominalCTscanseries(4,5).Infact,theincidenceofadrenalnod-ulesatautopsyisashighas32%amongpatientswithoutpremortemsu…     

Clinics in diagnostic imaging (39). Conn's syndrome due to adrenocortical adenoma (Conn's tumour)

A 33-year-old man presenting with muscle weakness and hypertension was found to have hypokalaemia and metabolic alkalosis. Computed tomography (CT) showed an adrenal mass. Conn's syndrome due to an adrenocortical adenoma was confirmed at surgery. Hypertension-related adrenal disorders such as Cushing's syndrome, carcinoma and phaeochromocytoma are discussed. CT is currently the single most useful imaging method for identifying adrenal masses.     

Adrenal insufficiency after operative removal of apparently nonfunctioning adrenal adenomas

We describe a woman who developed adrenal insufficiency after removal of an apparently nonfunctional adrenal adenoma. She displayed no stigmata of Cushing's syndrome and had normal plasma and urinary cortisol levels. A second patient without clinical findings of Cushing's syndrome also had normal basal steroid levels. This patient displayed partial suppressibility with dexamethasone, had low-normal levels of serum corticotropin, and excreted a low concentration of urinary 17-ketosteroids. She also developed mild adrenal insufficiency after the operation. We believe the adrenal adenomas in these patients secreted enough cortisol to suppress the contralateral adrenal gland but not enough hormone to elevate basal steroid levels. Therefore, we suggest that all patients with adrenal masses be studied with the overnight dexamethasone suppression test rather than basal steroid hormone measurements to detect low levels of autonomous cortisol secretion. In addition, patients with adrenal masses that are not removed surgically should have serial adrenal function tests performed.     

CLINICAL USE OF 131I-19-IODOCHOLESTEROL ADRENAL SCINTIPHOTOGRAPHY

51 cases of adrenal disease were diagnosed by 131I_19_iodocholesterol adrenal scintiphoto- graphy. Of these, 40 were primary aldosteron- ism and 11 Cushing's syndrome. 32 0f the 40 cases of primary aldosteronism lvere operated ripori througli incision on the side with higher radioactivity. Adenomas were found in 26, hyperplasia in 5 and normal histology in l. Re troperitoneal oxygen insafflation performed in 21 cases derrtonstrated an enlarged adrenal gland in 3. Scintiphotography is of great clinical value in Iocalizing adrenal cortical adenoma in pri- mary aldost'eronism antl Cushing's syndrome.     

亚临床及肾上腺腺瘤型库欣患者的糖代谢紊乱

目的评价亚临床和肾上腺腺瘤型库欣患者糖代谢紊乱状况。方法对32例亚临床库欣(组1)、33例典型的肾上腺腺瘤型库欣(组2)及40例无功能瘤患者(组3)分别行口服糖耐量试验(OGTT),计算胰岛素敏感指数(ISI)、HOMA指数(HOMA-IR)、葡萄糖及胰岛素曲线下面积(AUCg,AUCi);同时检测各组患者血和尿皮质醇、血促肾上腺皮质激素(ACTH)及血钾等指标。以58名健康体检者作为对照组(组4)。结果组1、组2、组3和组4糖代谢紊乱的患病率分别为46.9%、48.5%、27.5%和22.4%;组2患者糖代谢相关指标,如空腹及糖负荷2 h后血糖(FBG及2 h PBG)、空腹及糖负荷2 h后血胰岛素(FIN及2 hPIN)及根据OGTT结果计算的AUCg、AUCi及HOMA-IR均高于其他组,而ISI则低于其他组别;剔除性别、年龄、腰围等相关因素的影响,多元回归分析结果显示,FBG、2 h PBG、AUCi与血、尿皮质醇水平呈正相关。组1患者皮质醇分泌仅为轻度升高,尚不足以引起库欣综合征典型的临床表现,但糖代谢紊乱和糖尿病的患病率亦升高(15.6%),其FBG、2 h PBG及AUCi等与血、尿皮质醇水平亦相关。结论亚临床及肾上腺腺瘤型库欣患者糖代谢紊乱的患病率明显升高,可能与皮质醇的高分泌状态有关。目的评价亚临床和肾上腺腺瘤型库欣患者糖代谢紊乱状况。方法对32例亚临床库欣(组1)、33例典型的肾上腺腺瘤型库欣(组2)及40例无功能瘤患者(组3)分别行口服糖耐量试验(OGTT),计算胰岛素敏感指数(ISI)、HOMA指数(HOMA-IR)、葡萄糖及胰岛素曲线下面积(AUCg,AUCi);同时检测各组患者血和尿皮质醇、血促肾上腺皮质激素(ACTH)及血钾等指标。以58名健康体检者作为对照组(组4)。结果组1、组2、组3和组4糖代谢紊乱的患病率分别为46.9%、48.5%、27.5%和22.4%;组2患者糖代谢相关指标,如空腹及糖负荷2 h后血糖(FBG及2 h PBG)、空腹及糖负荷2 h后血胰岛素(FIN及2 hPIN)及根据OGTT结果计算的AUCg、AUCi及HOMA-IR均高于其他组,而ISI则低于其他组别;剔除性别、年龄、腰围等相关因素的影响,多元回归分析结果显示,FBG、2 h PBG、AUCi与血、尿皮质醇水平呈正相关。组1患者皮质醇分泌仅为轻度升高,尚不足以引起库欣综合征典型的临床表现,但糖代谢紊乱和糖尿病的患病率亦升高(15.6%),其FBG、2 h PBG及AUCi等与血、尿皮质醇水平亦相关。结论亚临床及肾上腺腺瘤型库欣患者糖代谢紊乱的患病率明显升高,可能与皮质醇的高分泌状态有关。     

双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征3例临床诊疗探索

目的 探讨双侧肾上腺高功能皮质腺瘤致促肾上腺皮质激素（ACTH）非依赖性库欣综合征的临床特点、诊治思路,提高对该疾病的认知。 方法 收集2016～2021年四川大学华西医院内分泌代谢科收治的3例双侧肾上腺功能性皮质腺瘤致ACTH非依赖性库欣综合征患者行回顾性分析,分析患者的一般情况、症状及体征、辅助检查、诊治过程等临床资料,并结合文献进行复习和讨论。 结果 3例患者均表现为典型且严重的库欣综合征,并伴有显著的心脏和代谢并发症,影像学提示双侧肾上腺单发腺瘤;2例患者行双侧肾上腺静脉采血,均提示双侧肾上腺皮质醇高分泌且无优势侧;1例患者行双侧肾上腺肿物切除术,另外2例患者因为心功能不全严重,行分期双侧肾上腺肿物切除术,术后病理结果3例均提示双侧肾上腺皮质腺瘤,术后皮质醇浓度均明显降低,均给长期予糖激素替代治疗。 结论 双侧肾上腺高功能皮质腺瘤致ACTH非依赖性库欣综合征非常罕见,明确诊断并判断双侧功能定位非常重要,治疗上应根据患者的病情及并发症情况个体化的选择最适合患者的手术方式,术后应严密随访,及时给予并调整糖皮质激素替代治疗,并终身随访。     

血浆ACTH测定在柯兴氏综合征鉴别诊断中的价值

本文报告经手术、病理证实的24例柯兴氏综合征患者血浆 ACTH 水平的改变,并与大剂量地塞米松抑制试验作了比较。结果表明17例柯兴氏病患者中14例血浆 ACTH(8am)水平增高,3例正常高限,0 am ACTH 水平全部增高,且无昼夜节律变化;6例肾上腺皮质腺瘤血浆ACTH 均明显降低;1例异位 ACTH 综合征血浆 ACTH 异常增高。三组之间无一例重叠,与手术、病理的符合率为100%。大剂量地塞米松抑制试验的符合率为67～70.6%。提示血浆 ACTH 测定是鉴别柯兴氏综合征病因十分可靠的方法。     

库兴氏病的垂体放疗：附134例报告

１３４例库兴氏病患者，采用垂体放疗或肾上腺及垂体瘤术后加垂体放疗。照射使用６０钴远距离治疗机或直线加速器，总有效率达８ｌ．３％。并对库兴氏病与Ｎｅｌｓｏｎ氏综合征的疗效、垂体照射的最佳剂量与放射损伤等进行了讨论。     

Cushing's syndrome--transitory immune deficiency state?

A 28 year old female patient with Cushing's syndrome due to an adrenal adenoma also suffered from recurrent urinary infections (proteus), tonsillitis (streptococcus), permanent candidiasis and perimandibular abscess (Staphylococcus pyogenes). Suppression of cellular and humoral immunity was confirmed by in vitro tests. After successful right adrenalectomy the clinical signs of Cushing's syndrome disappeared and no evidence of either bacterial or fungal infection were noted one year postoperatively. Immunological tests showed the restitution of both cellular and humoral immunity. The course of the disease in the patient supports the idea that Cushing's syndrome might be considered as a transitory immune deficiency state.     

肾上腺皮质疾病53例外科诊治体会

目的:探讨肾上腺皮质疾病的定性、定位诊断及围手术期处理方法,提高肾上腺皮质疾病的术前确诊率及外科诊疗水平。方法:对53例肾上腺皮质疾病患者的临床表现、术中经过、术后病理及随访结果进行回顾性分析。结果:原发性醛固酮增多症皮质腺瘤20例、柯兴综合症皮质腺瘤15例、无功能肾上腺皮质瘤8例,经手术后5年随访均已治愈,未见复发;肾上腺皮质结节样增生6例,其中左侧增生3例、右侧增生1例,双侧增生2例。4例作患侧肾上腺全切除,术后1年随访其临床症状及体征均有好转;2例作左侧肾上腺全切除、右侧肾上腺大部切除,术后出现肾上腺皮质机能危象,1年后随访出现Nelson综合征,需要补充肾上腺皮质激素。肾上腺皮质癌及肾上腺皮质转移癌4例,术后1年2例因恶性程度高发生转移而死亡,另2例在5年内死亡。结论:良性肾上腺皮质肿瘤手术效果及预后满意;恶性肾上腺皮质肿瘤应早期确诊、及时手术探查以提高手术疗效;肾上腺皮质疾病除选择正确的手术方案外,还应配合适当的围手术期处理以保证手术经过顺利。