静息型促肾上腺皮质激素腺瘤合并类肢端肥大症一例报道并文献复习

静息型促肾上腺皮质激素腺瘤(silent corticotroph adenoma,SCA)合并类肢端肥大症非常少见,国内外也无相关病例报道.但其极易误诊为肢端肥大症,因为该患者既有肢端肥大症的临床表现,又有垂体瘤的影像学改变,使人很容易想到生长激素(GH)瘤引起肢端肥大症.     

静息型促肾上腺皮质激素腺瘤合并类肢端肥大症一例报道并文献复习

静息型促肾上腺皮质激素腺瘤(silent corticotroph adenoma,SCA)合并类肢端肥大症非常少见,国内外也无相关病例报道.但其极易误诊为肢端肥大症,因为该患者既有肢端肥大症的临床表现,又有垂体瘤的影像学改变,使人很容易想到生长激素(GH)瘤引起肢端肥大症.     

肢端肥大症性心脏病1例报告并文献复习

<正>肢端肥大症是因垂体肿瘤过度分泌生长激素(GH)所致的、以肢体和器官肥大为特征的一种内分泌疾病。病程超过10年以上的肢端肥大症患者几乎都会发生心血管系统病变,后者是导致肢端肥大症患者死亡的最主要原因,约80%未经治疗的肢端肥大症患者在60岁以前死于各种心血管系统并发症。肢端肥大症患者常因各种心血管疾病症状如胸闷气短等就诊于心血管科,被误诊为冠心病、高血压性心脏病等。本例患者曾多次被误诊为"冠心病"。     

老年肢端肥大症合并垂体微腺瘤卒中一例

患者女性,82岁.因阵发性头痛1周,加重伴恶心、呕吐于2006年1月15日入院.患者曾于1998年因面容改变及手足指端粗厚、血生长激素(GH)增高、垂体MRI检查显示为垂体微腺瘤,确诊为肢端肥大症、垂体生长激素瘤.因顾虑年龄大及手术和放疗的风险,患者选择溴隐停治疗,15mg/d,分次口服,血GH一直稳定于20 mg/L,8年内定期复查垂体MRI,肿瘤体积无明显增大.     

肝脏转移性类癌合并肢端肥大症1例

患者女,60岁。因面部发红、心悸、全身乏力,伴大关节疼痛4个月于1982年2月23日入院。查体:T 37.5℃,P 72/分,R 20/分,BP 131/70mmHg。颜面微红略肿,头颅宽大,前额及下颌突出,鼻粗大,唇厚,舌大而厚。心肺正常。肝右肋     

男性垂体促性腺激素腺瘤5例报道并文献复习

报道了5例成年男性垂体促性腺激素腺瘤,均为无功能腺瘤,以视物模糊为主要症状就诊,肿瘤直径2～4 cm,血清促性腺激素水平均未见明显变化.患者术后随访1～3年,均未见肿瘤复发.     

肢端肥大症性心肌病1例报道

正1 资料病人,男,33岁,因反复胸闷、憋喘半年余,加重3 d入院。病人诉近半年来,小于日常体力活动时出现胸闷、憋喘不适,无胸痛、放射痛,无咳嗽、咳痰,无心悸、出汗,无呼吸困难、发绀、蹲踞现象,休息数分钟可缓解,未予以重视,此后逐渐出现静息状态下的胸闷、憋喘,遂就诊于当地医院,心脏超声提示:左室舒张内径71 mm,左房前后径35 mm,左室顺应性减低,二尖瓣、三尖瓣关闭不全(轻度);心电图提示:窦性心动过速,心率113次/min,偶     

老年类肢端肥大症伴肢端发绀症1例

肢端发绀症属血管性皮肤病,以肢端肥大而就诊者临床少见。我院收治１例类肢端肥大症伴肢端发绀症患者,现报告如下。患者女,６８岁。幼年时手脚即比常人粗大,但无感觉异常。近６年来手足逐渐增宽、增大,且双手渐感觉麻木,遇寒冷刺激后手指呈对称性紫绀,尤其是遇冷水刺激后双手发绀,发凉,伴有冷汗,手指有明显肿胀麻木感,温暖后可缓解;颜面部外观无改变。３０年前患骨结核致下肢髋关节畸形,跛行。查体：Ｂｐ１８／１０ｋＰａ。无明显肢端肥大症外貌,下颌无前突。皮肤粗厚,心肺听诊无异常,肝脾无肿大,双下肢不肿,双足趾跖无明…     

肢端肥大症性心脏病一例

1临床资料 男性患者，45岁。因活动后气促、喘憋2个月，加重2周入院。患者2个月前出现活动后气促、喘憋，当地医院心脏超声心动图示：全心弥漫性扩大，二尖瓣大量返流，左室射血分数（EF）20％，诊断为扩张性心肌病。近2周患者喘憋阵发加重，夜间不能平卧，于当地医院经抗心力衰竭治疗后症状缓解不明显，为求进一步诊治转入我院。既往无高血压、冠心病、瓣膜性心脏病及心肌炎病史，无嗜酒。入院查体：血压105／60mmHg（1mmHg=0．133kPa），呼吸22次／min，神志清，端坐位，皮肤粗糙，唇略发绀、增厚，耳鼻及下颌增大，颧弓突出，眉弓高，四肢指（趾）间关节短粗；双肺底可闻及少量小水泡音；心界向左扩大，心率89次／min，早搏3～6次／min，心尖部可闻及3／6级收缩期杂音，向腋下、后背部传导。     

垂体生长激素瘤合并甲状腺高功能腺瘤一例报告并文献复习

生长激素瘤合并甲状腺高功能腺瘤临床较为罕见,本文报道1例垂体生长激素瘤合并甲状腺高功能腺瘤患者的临床特点、治疗、转归,并对两者关系的近期国内外研究进行复习.     

Predictors of silent corticotroph adenoma recurrence; a large retrospective single center study and systematic literature review

Purpose

Silent corticotroph adenomas (SCAs) are clinically silent and non-secreting, but exhibit positive adrenocorticotropic hormone (ACTH) immunostaining. We characterized a single center cohort of SCA patients, compared the SCAs to silent gonadotroph adenomas (SGAs), identified predictors of recurrence, and reviewed and compared the cohort to previously published SCAs cases.

Methods

Retrospective review of SCA and SGA surgically resected patients over 10 years and 6 years, respectively. Definitions; SCA—no clinical or biochemical evidence of Cushing’s syndrome and ACTH positive immunostaining, and SGA—steroidogenic factor (SF-1) positive immunostaining. A systematic literature search was undertaken using Pubmed and Scopus.

Results

Review revealed 814 pituitary surgeries, 39 (4.8%) were SCAs. Mean follow-up was 6.4 years (range 0.5–23.8 years). Pre-operative magnetic resonance imaging demonstrated sphenoid and/or cavernous sinus invasion in 44%, 33% were >?50% cystic, and 28% had high ACTH levels pre-operatively. Compared to SGAs (n?=?70), SCAs were of similar size and invasiveness (2.5 vs. 2.9 cm, p?=?0.2; 44 vs. 41%, p?=?0.8, respectively), but recurrence rate was higher (36 vs. 10%, p?=?0.001) and more patients received radiation therapy (18 vs. 3%, p?=?0.006). Less cystic tumors (0 vs. 50%, p?<?0.001) and higher pre-operative ACTH levels (54 vs. 28 pg/ml, p?=?0.04) were predictors of recurrence for SCAs.

Conclusion

This review is unique; a strict definition of SCA was used, and single center SCAs were compared with SGAs and with SCAs literature reviewed cases. We show that SCAs are aggressive and identify predictors of recurrence. Accurate initial diagnosis, close imaging and biochemical follow up are warranted.

     

胰升糖素瘤:一例报告并文献回顾

患者男性,42岁,因"全身皮肤破溃5年,加重3个月余"于2009年9月3日入院.5年前出现全身皮疹,伴瘙痒、糜烂,下肢、会阴部尤为明显,曾在外院行皮肤病理活检诊为"糠秕孢子菌性毛囊炎",予酮康唑膏、皮康王膏、中草药等外敷治疗,症状稍有缓解,但时常反复,迁延不愈,伴纳差、乏力、消瘦,近3个月来伴舌痛.起病以来无腹痛、腹泻.精神睡眠尚可,饮食欠佳,体重明显减轻,大小便正常.入院体格检查:身高166cm,体重50 kg,体温36.5℃,心率77次/min,血压120/75mmHg(1 mm Hg=0.133 kPa).面部、躯干、腹股沟、四肢散在红斑,大片褐色痂皮,右下肢有大疱,双足底角化、脱屑(图1).全身皮肤黏膜无黄染,浅表淋巴结无肿大,心肺无异常,腹平软,肝脾肋下未触及,未触及明显包块.血常规血红蛋白(Hb)112g/L.血钾2.78 mmol/L.肝肾功能、心肌酶均正常.血清总蛋白57.4 g/L,白蛋白21.21g/L.口服葡萄糖耐量试验(OGTT):空腹血糖6.43 mmol/L,餐后2h血糖9.65 mmol/L,HbA1c 5.7％.心电图、胸片未见异常.腹部彩超未见异常.     

伴促甲状腺激素瘤的多发性内分泌腺瘤病1型一例报道及文献复习

目的通过研究1例伴有促甲状腺激素瘤的多发性内分泌腺瘤病1型(MEN1)患者的临床诊治过程及结局以提高对本病的认识。方法分析1例伴有促甲状腺激素瘤的MEN1患者诊治过程中的临床表现、生化和激素、影像学、手术及术后病理结果。使用二代测序再以Sanger法验证分析MEN1及其他基因,并以PolyPhen2和PROVEAN在线检测其产物危害性。结果1例19岁的男性患者因高代谢症状和甲状腺激素(THs)升高诊断甲亢,TSH 2.78 mIU/L,TSH受体抗体(TRAb)阴性。并发现高血钙和低血磷、血浆甲状旁腺激素(PTH)升高以及右下甲状旁腺99mTc显影阳性,诊断甲状旁腺腺瘤,手术治疗后上述指标恢复正常。抗甲状腺药物治疗1年症状和激素无明显改变,MRI显示右下垂体大腺瘤,考虑TSH瘤;经鼻蝶入路切除肿瘤后1个月复查TSH和TH恢复正常,免疫组化TSH阳性。CT和MRI发现胰腺体尾部占位,无相关症状。基因分析发现MEN1杂合突变:c.415C>T,p.His139Tyr(H139Y),蛋白质预测为有害。文献复习目前仅有5例报道。结论MEN1中的TSH瘤少见,既需要在甲状腺功能亢进中鉴别出TSH瘤,同时要考虑TSH瘤合并于MEN1,以减少误诊误治。     

Thyrotropin-secreting adenoma in multiple endocrine neoplasia type 1:one case report and literature review

正Objective To improve the understanding of thyrotropin-secreting adenoma in multiple endocrine neoplasia type 1 (MEN1) through analyzing the clinical diagnosis and treatment process,as well as outcomes in one case of this disorder.Methods The clinical manifestations,biochemical and hormone levels,imaging presentations,medical and surgical treatments,and post-operational     

Large Brunner＇s gland adenoma： Case report and literature review

Brunner's gland adenoma(BGA)is a very rare benigntumour of the duodenum,which is usually asymptomaticand discovered incidentally at endoscopy.Occasionally,this lesion may be large,causing upper gastrointestinalhaemorrhage or intestinal obstruction.The case had alarge Brunner's gland adenoma,presenting melena thatwas managed by endoscopic excision.     

脾梗死1例报道并文献复习

在内科诊疗工作中,脾梗死较罕见,易漏诊。本文报道1例长期发热患者,通过对其脾梗死原因的探索理清其长期发热的原因;并通过复习相关文献,加强对脾梗死的原因、临床表现及诊疗等的认识。