Role of adrenalectomy in recurrent Cushing’s disease

Background Cushing＇s disease is a pituitary-dependent type of Cushing＇s syndrome. Treatment consists of pituitary surgery or radiotherapy, but the recurrence rate at 10 years is as high as 40%. Adrenalectomy is considered an effective treatment to refractory Cushing＇s disease. The objective of this study was to examine the efficacy of laparoscopic adrenalectomy and open adrenalectomy in Cushing＇s disease, focusing on reversing the sequelae of hypercortisolism and improving patients＇ quality of life. Methods Forty-three patients （29 women, 14 men） with recurrent Cushing＇s disease after transsphenoidal operation underwent laparoscopic （n=32） or open （n=11） adrenalectomy from 2000 to 2008. Surgical results were evaluated for all the 43 patients. Patients completed a follow-up survey, including the short-form 36-item （SF-36） health survey. Results All the 43 patients achieved clinical reversal of hypercortisolism after adrenalectomy. Time to symptom resolution varied from a few weeks to up to 3 years. Most physical changes had resolved by a mean of 8 months after surgery. These conditions were not significantly different between the laparoscopy and open groups. Median length of hospital stay was shorter in the laparoscopy group （4 vs. 9 days; P 〈0.001）. Median follow-up was 48.5 months. Of the 34 （79%） patients available for follow-up, 22 （65%） had adrenocorticotropic hormone levels 〉200 ng/ml and 6 （27%） had clinical Nelson syndrome. Four patients died by 75 months after surgery. Using SF-36, 30 （88%） patients reported they felt their health status was good to excellent compared with 1 year before adrenalectomy; however, they showed significantly lower scores in all the 8 SF-36 parameters compared with the general population. No significant difference emerged in SF-36 scores between the laparoscopy and open groups. Conclusions Adrenalectomy showed high survival and clinical benefits in recurrent Cushing＇s disease patients. Despite patient-reported improvement in health after adrenalectomy, patients continue to experience poor health status compared with the general population.     

Blue rubber bleb nevus syndrome: a report of one case

Blue rubber bleb nevus syndrome （BRBNS）, or Bean＇s syndrome, is a rare angiomatosis characterized by distinctive cutaneous and gastrointestinal venous malformations that usually cause massive or occult gastrointestinal hemorrhage and iron deficiency anemia secondary to the bleeding episodes. Other organs may also be involved. We report a case with symptoms of recurrent epistaxis and chronic gastrointestinal hemorrhage. We hope to emphasize the possibility of recurrent epistaxis involvement in BRBNS and heighten physicians＇ awareness of the disease to contribute its early detection.     

Radiotherapy of ectopic ACTH syndrome due to thoracic carcinoids.

From 1984 to 1990, six patients with histologically and endocrinologically proven ectopic ACTH syndrome were treated in our department. The lesion in five patients was clinically evident, but in the other was occult. All lesions were located within the chest. Four were thvmic carcinoid and the other two were bronchial carcinoids. Most of the patients had typical clinical manifestations of Cushing's syndrome. Laboratory tests including histopathological examination confirmed the diagnosis of ectopic ACTH syndrome. The treatment consisted of surgical removal of the mass supplemented by radiotherapy. The results showed that after treatment all patients had satisfactory remission. Two bronchial carcinoid patients have been living for 40 and 88 months respectively without recurrence. Among 4 thymic carcinoid patients, only one has been living with no tumor for 34 months. One patient had recurrence and the other 2 died 32 and 50 months after the treatment respectively. However, the symptoms of two thymic carci     

Microsurgical treatment of Nelson’s syndrome

Objective To discuss the etiology, diagnostic criteria and treatment of Nelson’s syndrome.Methods Twenty-three patients with Nelson’s syndrome who were treated in our department over the last 19 years were analyzed retrospectively.Removal of adenoma by the transsphenoidal approach was done in 21 patients and by transfrontal craniotomy in 2.The follow-up period ranged from six months to nine years.Results The incidence of Nelson’s syndrome was 7.7% in a series of 300 patients with Cushing’s disease treated by microsurgery in the same period.Hyperpigmentation was relieved and adrenocorticotropic hormone (ACTH) levels decreased in all patients after tumor excision.Eight patients with visual disturbance improved after surgery.The curative and remission rates were 56.5% and 26.1%, respectively.Conclusions Transsphenoidal microsurgical removal of pituitary ACTH adenoma is the first choice in the prevention and treatment of Nelson’s syndrome.Regular follow-up examinations should be performed over a long time.     

Severe crush syndrome complicated with acute pancreatitis: a case report and review of the literatures

Earthquake is one of the most catastrophic natural disasters. As we know, crush syndrome is the secondmost frequent cause of mortality after the direct impact of trauma.1-3 It is a serious clinical case that develops among casualties of earthquakes or other catastrophic events that may also result in a variety of ensuing uncommon complications. Cases of crush syndrome complicated with acute pancreatitis （AP） following the earthquakes are seldom reported.     

Imaging diagnosis of two unusual forms of gallstone ileus

Ileus from an impacted gallstone in the duodenum or any other part of intestine is a rare complication of cholelithiasis. When a gallstone impacted in the distal stomach or in the duodenum, it is called Bouveret＇s syndrome, a rare form of gallstone ileus. Small bowel ileus caused by gallstone has become more and more common recently due to popular usage of computed tomography （CT） and magnetic resonance imaging （MRI）. In the past, imaging diagnosis relied primarily on conventional radiography and contrast-enhanced fluoroscopy. CT has subsequently been shown to be quite useful for this diagnosis. Recently, MR cholangiopancreatography （MRCP） has emerged as a valuable noninvasive technique for evaluation of biliary disease. Here we described the CT, MRCP and gastroenterography findings in two patients with unusual form of gallstone ileus （GI）, one is Bouveret＇s syndrome and the other is negative gallstone-induced ileus. Few reports about GI with angiografin and MRCP findings of Bouveret＇s syndrome has been published.     

A case of Poncet’s disease: retroperitoneal tuberculous lymphadenitis and polyarthritis

To the editor： Reactive arthritis （ReA） in tuberculosis （TB） is known as Poncet＇s disease. It is a rare aseptic form of arthritis observed in patients with active TB. It has been suggested that Poncet＇s disease mainly occurs in patients with extrapulrnonary TB. We report here a case of Poncet＇s disease in a patient with retroperitoneal tuberculous lympbadenitis.     

Nasal continuous positive airway pressure combined with surfactant and NO for treatment of respiratory distress syndrome, prevention of bronchopulmonary dysplasia, and brain protection

Respiratory distress syndrome （RDS） is the single .most important cause of mortality and morbidity in preterm infants and bronchopulmonary dysplasia （BPD） is a leading cause of neuro-muscular disablement and decreased lung function in the most preterm infants.     

MAGNETIC RESONANCE IMAGING CHARACTERISTICS AND SURGICAL RESULTS OF ADRENOCORTICOTROPIN-SECRETING PITUITARY ADENOMAS

Objective To evaluate magnetic resonance imaging （MRI） characteristics and surgical results of adrenocorticotropin （ACTH） -secreting pituitary adenomas. Methods MRI characteristics and relationship between MRI positive rate and surgical results of 266 patients with pathologically confn＇med Cushing＇s disease were analyzed retrospectively. All patients underwent thin-section sagittal and coronal scans of the pituitary gland before and after administration of gadolinium-diethylene triamine pentaacetic acid （Gd-DTPA） on a 1. 5 Tesla MR/scanner, and dynamic enhanced MR/was performed in 39 patients. All patients underwent transsphenoidal adenomectomy. Endocrinological examinations and assessments were performed. Results Preoperative MR/revealed normal results in 41 （ 15.4% ） cases, microadenoma in 179 （67. 3% ）, macroadenoma in 42 （ 15.8% ）, and huge adenoma in 4 （ 1.5% ）. Pituitary apoplexy was found in 13 （4.9%） cases. Positive rate of ACTH-secreting adenomas was 84.6% （225/266） on MR/scans, and that of small microadenomas was 87.2% （34/39） on dynamic enhanced MRI scans. Preoperative endocrinological tests of 199 cases supported the diagnosis of typical Cushing＇s disease, while the other 67 cases had atypical endocrinological results. The endocrinological cure rate, remission rate, and inefficacy rate were 85.7%, 7.9%, and 6. 4%, respectively. There was no difference in the initial endocrinological cure rate between the patients with positive and normal MRI results （90% vs. 87. 8%, P =0. 904）. Conclusions Enhanced coronal pituitary MR/ is helpful for preoperative localization of ACTH-secreting pituitary microadenoma. Dynamic enhanced MR/may improve detection rate of microadenoma. There is no marked difference in the surgical results for patients with preoperative MR/results indicating presence or absence of microadenoma.     

High prevalence of obstructive sleep apnea in Marfan’s syndrome

Objective To review the current evidence about the prevalence of obstructive sleep apnea in patients with Marfan＇s syndrome,and discuss some proposed potential mechanisms for this relationship.Data sources The data in this review were mainly from Medline and PubMed articles published in English from 1990 to 2013.The search term was ＂Marfan＇s syndrome and sleep apnea＂.Study selection Clinical evidence about the epidemiology of obstructive sleep apnea in patients with Marfan＇s syndrome; the mechanism that causes obstructive sleep apnea; interventional therapy for patients with Marfan＇s syndrome,and coexisting obstructive sleep apnea.Results A high prevalence of obstructive sleep apnea exists in patients with Marfan＇s syndrome.The potential reasons are craniofacial abnormalities and lax upper airway muscles,which lead to high nasal airway resistance and upper airway collapse.Obstructive sleep apnea mechanically deteriorates aortic dilatation and accelerates progression of aortic aneurysms.The condition is reversible and rapid maxillary expansion and adequate continuous positive airway pressure therapy are possible effective therapies to delay the expansion of aortic diameter in patients with Marfan＇s syndrome.Conclusions Obstructive sleep apnea is strongly associated with Marfan＇s syndrome.Craniofacial abnormalities and lax upper airway are the main mechanisms.Untreated obstructive sleep apnea accelerates progression of aortic dissection and rupture.Effective therapies for obstructive sleep apnea could postpone the aortic dilatation in patients with Marfan＇s syndrome.     

唐氏综合征伴发急性白血病的诊治

1病历摘要 患儿,男孩,1岁7个月,因“颜面苍白伴皮下出血点1周”于2013年2月入院。 1周前家属发现患儿颜面明显苍白,全身皮肤可见散在出血点,无鼻出血、牙龈出血,口唇少许溃疡,无发热,无呕吐,无夜间盗汗,无排柏油样便,小便正常。     

骨髓坏死综合征骨髓象的分析

骨髓坏死综合征是由多种原发病引起的一种不常见的骨髓内死亡。预后很差,其诊断主要由骨髓穿刺图片和骨髓组织切片活检证实。现将我院遇到的11例病例报道如下。1临床资料1.1一般资料11例骨髓坏死均为我院1990年至1998年门诊或者住院患者。其中男9例,女2例,年龄30岁~65岁。1.2临床表现11例患者中7例发烧,8例贫血,11例有骨痛,8例肝脾肿大,3例淋巴结肿大。11例患者中有4例骨髓转移癌,1例阴道糜烂4个月,1例鼻腔有肿块放疗3次好转,1例腋下肿块2a,1例胃癌,多发性骨髓瘤2例,急性白血病1例。1.3实验室检查外周血检查:在11例患者的外周血涂片中有6例见有数量不等的幼稚粒细胞和幼稚红细胞。骨髓检查:全部病例经骨髓穿刺取片黄位粒样物质,作瑞氏染色进行全片观察,其坏死的特征为:细胞呈不同程度溶解,胞浆无法辨认,细胞轮廓不清,呈紫血片状或蛛网状结构,细胞间充满无定性嗜酸性物质,坏死的细胞包括固缩、核破裂、核溶解,坏死的骨髓中有成熟红细胞,少数幼红细胞和巨核细胞。坏死累及范围大小不一。其坏死呈局灶性,视为轻度坏死;有的坏死范围占据整个标本的60%左右,视为中度坏死;有的坏死范围可占整个涂片,坏死呈大片,根本无...     

Effects of tacrolimus and cyclosporine treatment on metabolic syndrome and cardiovascular risk factors after renal transplantation: a meta-analysis

Background The therapeutic success of renal transplantation has been largely attributable to the development of effective and balanced immunosuppressive treatment regimens.This study provides a meta-analysis of a series of randomized controlled trials that compared the effects of tacrolimus and cyclosporine on metabolic syndrome （MetS） and cardiovascular risk factors after renal transplantation.Methods We searched various electronic databases and bibliographies,including MEDLINE,the Cochrane Central Register of Controlled Trials,and EMBASE,for relevant studies published prior to October 2012.Results Our meta-analysis included five randomized controlled trials that examined a total of 923 patients.The tacrolimus group and the cyclosporine group exhibited no significant differences in MetS incidence after renal transplantation; risk ratio （RR）：1.06,95％ confidence interval （C/）：0.73-1.55,P=0.76.Cyclosporine treatment was associated with a higher incidence of hyperlipidemia （RR：0.50,95％ CI：0.39-0.64,P ＜0.01）.Although there were no statistically significant differences,cyclosporine treatment was associated with a higher incidence of hypertension （RR：0.91,95％ CI：0.83-1.00,P=0.06） after renal transplantation compared to tacrolimus treatment,and tacrolimus treatment was associated with a higher incidence of diabetes after renal transplantation （RR：1.79,95％ CI：0.98-3.27,P=0.06） compared to cyclosporine treatment.Conclusions Compared to tacrolimus treatment,cyclosporine treatment was associated with a higher incidence of hyperlipidemia.Future large-scale studies are expected to be conducted to further confirm our findings.     

胡志敏教授运用经方辨证治疗放射性肺炎的经验总结

胡志敏教授是辽宁中医药大学第一附属医院硕士研究生导师，他从事教学、临床与科研工作20余年。擅用经方治疗各种放化疗并发症。并且有其独到之处。现将胡志敏教授辨证治疗放射性肺炎的经验介绍如下。     

血清脂蛋白(a)对冠心病与糖尿病及肾病等多种疾病的临床意义

血清脂蛋白Lp(a)水平升高与冠心病、糖尿病、肾病及多种疾病密切相关,血清Lp(a)水平升高与降低具有重要的临床意义。1Lp(a)与冠心病脂蛋白脂酶通过影响血脂代谢等途径在冠心病发生、发展中起着重要作用,充分认识有关机制可以提高冠心病及合并脂质代谢异常的干预和策略制定的水平。动脉粥样硬化(AS)是冠心病的重要病理基础。一般认为,血清Lp(a)浓度受基因控制与遗传有关,不受年龄、性别、营养和其他环境影响。Lp(a)浓度个体差异很大,但对同一个体其Lp(a)测定值相对恒定,其血清含量几乎无生理变化。Lp(a)作为AS和血栓性疾病的危险因子已越来越被人们所关注,已有研究证明,Lp(a)是一种独立的特异脂蛋白,血清中Lp(a)水平升高与冠心病等心脑血管疾病密切相关。Lp(a)是由载脂蛋白(a)和载脂蛋白B100通过二硫键结合而成的脂类中分子化合物。有许多资料都证实了Lp(a)具有致AS和促进血栓形成的作用。研究结果显示,冠心病患者Lp(a)水平明显高于对照组,提示Lp(a)在冠状动脉病变中起重要作用。冠心病患者中冠脉狭窄支数或分数越高,Lp(a)浓度越高,二者呈正相关,提示Lp(a)水平升高,可作为冠心病严重程度的预...     

动态心电图在病态窦房结综合征诊断中的意义

<正>病态窦房结综合征是心源性昏厥原因之一,也是心血管比较常见的严重疾病,主要是由于窦房结器质性病变或功能障碍,造成起搏和传导功能失常,产生多种心律失常的综合表现,甚至可以引起心脏性晕厥和猝死。动态心电图的问世     

Evaluation of triple anti-platelet therapy by modified throm- belastography in patients with acute coronary syndrome

Most cases of acute coronary syndrome （ACS） involve coronary atherosclerosis and plaque rupture, as well as subsequent thrombosis. The initial thrombotic events leading to red thrombus formation are platelet adherence and aggregation. Platelets play a very important role during the establishment and progression of a thrombosis in a coronary artery. Therefore, in recent years guidelines have been developed in an effort to strengthen antiplatelet therapy in ACS, but unfortunately the methods to evaluate platelet activity and the strength of platelet inhibition are lacking.     

Tumor Susceptibility in Proteus Syndrome:a Case Report

PROTEUS syndrome is characterized by patchy and progressive overgrowth affecting multiple tissues, including bone, soft tissue, and skin, along with susceptibility to the development oftumors. It was originally described by Cohen and Hayden in 19791 and named by Wiedmann in 1983.2 The cause of Proteus syndrome is proposed to be a somatic mutation that is lethal in non-mosaic state; cells derived from the mutated cell line carrying this mutation result in anomalies in multiple tissues.3 The clinical manifestations are variable,     

Application of immunosuppressant facilitates the therapy of optic neuritis combined with Sj（o）gren＇s syndrome

Background Optic neuritis （ON） is often the first symptom of multiple sclerosis （MS） and neuromyelitis optica （NMO） while there has been very little research reported on ON combined with Sj（o）gren＇s syndrome （SS）.The aim of this study is to provide different treatments and services for and NMO patients combined with SS.Methods Twenty-seven patients with ON combined SS were divided into two groups：corticosteroid group （C group,methylprednisolone sodium succinate,14 patients） and corticosteroid＋ immunosuppressant group （C＋I group,leflunomide,13 patients）.ON relapse times in 1 year after treatment,number of patients who relapsed to NMO/MS in 1 years,visual acuity and retina nerve fiber layer （RNFL） thickness were measured.Mann Whitney-Wilcoxon test was used to compare continuous variables and Chi-square test or Fisher＇s exact test was to compare proportions.Results ON combined with SS patients had higher incidence rates in middle-aged women who have binocular damage and heavier visual function damage or when there is an easy relapse,and the patients are often hormone dependent.The patients are more likely anti-aquaporin-4 IgG seropositive （70.4％）.They are liable to form a centrocecal scotoma and tubular vision.The times of relapse decreased in patients who used immunosuppressant,and a significant difference was found between immunosuppressant and non-immunosuppressant groups in visual acuity recovery during 6-month followup period （P ＜0.05）; however,the RNFL thickness at the four quadrants was not significantly different.Conclusions The effect of immunosuppressant plus corticosteroid on the early onset of ON combined with SS was to provide ON remedy and to prevent recurrence in clinics.This study provides a significant reference for the prevention and treatment of ON on the basis of immunosuppressant and corticosteroid.