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目的 探讨桥本病合并其他甲状腺疾病的诊治策略。方法 回顾性分析1984－1999年间收治33例桥本病合并其他甲状腺疾病资料。结果 全部病人均行手术治疗，术后病理检查结果：桥本病合并甲状腺腺瘤9例，合并结节性甲状腺肿17例，合并甲状腺机能亢进3例，合并甲状腺乳头状癌4例。术后6例出现甲状腺功能减退。结论 临床医师应重视对桥本病的认识。细针穿刺活检对于明确诊断有重要意义。甲状腺局部孤立实性结节是手术适应证。术中采用快速冰冻切片检查有助于确定手术方式，术中应注意尽量保留正常的甲状腺组织，以防形成甲状腺功能减低。     

Non-Dupuytren's disease of the palmar fascia

The typical Dupuytren's disease patient is of Northern European descent with bilateral progressive multiple digital contractures and is genetically predisposed, with a family history. Palmar fascial proliferations sometimes present as a different entity without the typical Dupuytren's disease characteristics. We identified 39 patients (20 women and 19 men) over a 4-year period with "Non-Dupuytren's palmar fascial disease", with unilateral involvement, without family history or ectopic manifestations. Twenty-three patients presented with unrelated complaints and were discovered, incidentally, to have the condition. In 28 patients, prior ipsilateral hand surgery or trauma precipitated the condition. Other related factors were diabetes mellitus and cardiovascular disease. Ten patients had skin tethering and subcutaneous thickening akin to Dupuytren's nodules and 29 had palmar fascial thickening into ill-defined pretendinous cords. The diseased tissue was in the line of the ring finger in 30 patients. The time from insult to onset of contracture averaged 3.6 months and from onset to follow-up averaged 5.3 years. The condition was non-progressive, or partially regressive, in 33 patients. Seven patients had operations for unrelated conditions and underwent simultaneous fasciectomy without recurrence. Environmental factors, especially trauma, surgery and diabetes, are important in the pathogenesis of Non-Dupuytren's palmar fascial disease, but these patients do not appear to be genetically predisposed for Dupuytren's disease. Typical Dupuytren's disease and Non-Dupuytren's palmar fascial disease are two clinical entities that run different courses and do not share a similar prognosis. This should be taken into account in future epidemiological and outcome studies.     

Multiple osteochondroses of bilateral knee joints

We experienced a patient with a combination of multiple osteochondroses: Blount's disease, bipartite patella, and Sinding-Larsen-Johansson disease in the left knee, and a combination of bipartite patella and Osgood-Schlatter disease in the right knee. The patient was a healthy, active 12-year-old boy with bilateral knee pain. He had been diagnosed with Blount's disease of the left tibia at 2 years of age, and had been treated with open wedge osteotomy. He was diagnosed with bilateral bipartite patellae at the age of 9 years, and was diagnosed with Osgood-Schlatter disease in the right knee and Sinding-Larsen-Johansson disease in the left knee at 10 years of age. The second growth spurt was observed during this period. At 11 years of age, he was diagnosed with an osteochondral fracture of the left lateral femoral condyle and was observed without surgery. This patient showed the sequential appearance of an ossification disorder, probably due to the abnormal response of enchondral ossification to mechanical stress. Overuse in this growth period may have played a role in the development of these osteochondroses. The osteochondral fracture was probably caused by a disruption at one of the weakest parts of the developing skeleton, between the ossification center and the overlying cartilage in the background of an ossification disorder. Received: August 10, 2000 / Accepted: January 31, 2001     

A case of Moyamoya disease with progressive involvement from unilateral to bilateral

A case of Moyamoya disease in a child starting with unilateral lesion and developing into bilateral involvement is reported. Angiographic findings at onset showed unilateral involvement, hence, it was filed as a “probable” case according to the diagnostic criteria of the Japanese Cooperative Research Committee. The carotid angiograms on the other side were totally normal. Three years later the occlusive lesion became bilateral, to meet the criteria as a “definite” case. Clinical manifestations, angiograms, electroencephalograms, and position emission tomograms in this case are presented, and the relation between the probable and definite cases is discussed.     

Should patients with erectile dysfunction be evaluated for cardiovascular disease?

The landmark Massachusetts Male Ageing Study shed new light on the prevalence of erectile dysfunction (ED) and drew attention to ED as a disease of ageing. Over the years, ED has been linked to the development of cardiovascular disease (CVD) in some patients. There is clear evidence that ED and CVD share and have a similar risk factor profile. CVD is one of the most recognizable causes of mortality and early detection coupled with prevention of mortality from CVD has been the prime interest of many researchers. Consequently, there has been a multidisciplinary curiosity regarding the proposal to use ED as a marker for future CVD. In fact, there have been several proposals to use ED as a screening tool for future CVD. We performed a comprehensive search of two main databases-PubMed and Cochrane Library using a combination of keywords such as acute myocardial infarction, coronary artery disease (CAD) and ED. Journal articles from January 2000 to June 2011 were reviewed. We included all articles discussing the relationship between ED and CVD in the English language. All the relevant randomized controlled trials, cohort and retrospective studies, and review articles were included in our overall analysis in an attempt to answer the question whether all patients with ED should be clinically evaluated for CVD. The results showed a link between ED and the development of future CVD in some patients, but ED was not shown to be an independent risk predictor that is any better than the traditional Framingham risk factors. Screening for CVD may, however, be rewarding in younger patients with severe ED and in patients with concurrent CVD risk factors.     

Wide local excision and split-thickness skin graft for circumferential Paget's disease of the anus

BACKGROUND: Our aim was to evaluate the results of wide local excision followed by split-thickness skin graft for circumferential perianal Paget's disease. METHODS: Between 1995 and 1999, 5 patients with perianal Paget's disease underwent wide local excision of the disease. The circumferential involvement was documented by preoperative mapping. Standard orthograde bowel cleansing and oral antibiotics were given the day prior to surgery. The wound was allowed to granulate and confirmation of negative margins was obtained on permanent sections taken from the excised specimens. On postoperative day 4, split-thickness skin grafts harvested from the lateral thigh were applied (surface using area ranging from 100 cm(2) to 240 cm(2)). The grafts were secured in place with a 3-0 Vicryl suture and covered with a pressure dressing. After 4 days of bedrest, the dressing was taken down under anesthesia (day 8). RESULTS: Graft survival was 100% in 3 patients, 80% in 1 patient, and 70% in another. Postoperatively, clinical follow-up was conducted at 3-month intervals. In 2 patients, anal stenosis developed after the operation and resolved by gentle self-dilation. At a mean follow-up of 42 months (range 21 to 78), adequate cosmetic and functional results were obtained. Recurrence of Paget's disease has occurred in 1 patient (at 21 months) and an invasive anal gland carcinoma was found in 1 patient (at 48 months). CONCLUSIONS: Wide local excision and circumferential split-thickness skin grafting achieves adequate cosmetic and functional results without the need for diverting colostomy. However, there is a considerable recurrence rate after surgery, which warrants very close follow-up to rule out recurrent disease or underlying adenocarcinoma.     

Paget病19例

目的 提高对Ｐａｇｅｔ病的诊治水平。方法 回顾性分析１９８７年至１９９７年收治的１９例Ｐａｇｅｔ病的临床表现及诊治方法。结果 本组乳腺Ｐａｇｅｔ病６例,乳腺外１３例,其中阴茎,阴囊１１例,腹股沟处１例,肛周肛管１例。随访时间０．５－１１年,平均５年４个月,随访中３例复发者,其截端切片病理检查均可见病变组织,另１例死于其他疾病。     

慢性肾脏病住院患者白蛋白尿与心血管疾病相关性研究

目的：研究慢性肾脏病（CKD）住院患者白蛋白尿与心血管疾病（CVD）的相关性,探讨白蛋白尿对非糖尿病CKD患者CVD的预测价值。方法：回顾性分析1245例非糖尿病CKD患者的一般情况、生化指标、心电图、胸部X线、心超及CVD的危险因素。结果：（1）1245例患者中CKD1、2、3、4、5期分别为304例（24.4%）、281例（22.6%）、372例（29.9%）、157例（12.6%）、131例（10.5%）;CKD1～5各期有蛋白尿者分别为208例（68.8%）、194例（69%）、269例（72.3%）、117例（74.5%）、106例（80.9%）。（2）与CKD1期患者相比,CKD2～5期患者年龄、SBP、DBP、Scr、UA明显升高,eGFR、Hb、Alb明显降低（P〈0.05）;CKD3期患者TG、LDL升高,HDL降低（P〈0.05）;CKD4、5期患者TC、LDL、HDL降低;TG升高（P〈0.05）。（3）与CKD同期非白蛋白尿组相比,白蛋白尿组CKD1～5期患者Scr、UA明显升高,Alb明显降低（P〈0.05）;CKD2～5期患者SBP、DBP明显升高,eGFR、Hb明显降低（P〈0.05）;CKD4、5期患者TC、HDL降低,TG、LDL升高（P〈0.05）。（4）CKD患者CVD发病率从CKD1～CKD5期逐步升高（P〈0.05）,白蛋白尿患者CVD发病率以及胸部X片、心电图、心超异常阳性率升高更加明显（P〈0.05）。（5）Logistic回归分析显示CVD与年龄、SBP、UA、TG、白蛋白尿呈现正相关,与GFR、Hb呈现负相关（P〈0.05）。结论：非糖尿病CKD患者CVD发病率随CKD进展而增高,与白蛋白尿密切相关,白蛋白尿是CVD患者心血管疾病危险标志。     

Isolated single coronary artery from nonadjacent sinus of Valsalva

We present the computed tomography angiogram images of a 43-year-old lady with an isolated single coronary artery arising from the nonadjacent sinus of aortic root.     

Surgeons should be wise when choosing a material to repair heart defects

Various types of prosthetic materials are available for usage in cardiac surgery to repair heart defects. PTFE, Dacron, and bovine pericardial are some examples of the most used materials for this purpose. In this article, the authors describe the potential failure of the CorMatrix®.     

Cystic adventitial disease: a case report and literature review

Cystic adventitial disease (CAD) is a rare non-atherosclerotic cause of peripheral vascular disease (PVD). We describe a 54-year-old man who presented with calf claudication and catheter angiography showed the typical scallop appearances of CAD. Surgery involved resection of the diseased segment with a femoral popliteal venous bypass graft. A literature review revealed the different theories for pathogenesis, the investigation of choice being catheter angiography and the treatment excision with bypass grafting.     

The co-existence of Fabry and celiac diseases: a case report

We present a patient with Fabry disease with remarkable diagnostic findings and gluten-sensitive enteropathy. An 11-year-old girl was admitted to hospital with weight loss, anorexia, nausea, vomiting, flank pain, acroparesthesia, and painful extremities. Her mother had end-stage renal failure secondary to Fabry disease. On physical examination, she had growth retardation. Ophthalmological examination showed characteristic whorl-like corneal opacities and Fabry disease was confirmed with low -galactosidase A (-gal A) activity. Her painful attacks were treated with carbamazepine, but vomiting and nausea continued. Laboratory studies revealed positive serum anti-endomysium and anti-gliadin antibodies. Small intestinal biopsy showed subtotal villous atrophy compatible with gluten-sensitive enteropathy. Following treatment with a gluten-free diet, her gastrointestinal symptoms completely disappeared within a few weeks and then she had catch-up growth. In her long-term follow-up, proteinuria appeared and renal involvement was confirmed by characteristic renal biopsy findings. Following these clinicopathological findings, enzyme replacement therapy was started. In conclusion, although heterozygous females can be asymptomatic or are expected to have a mild course of the disease, a severe clinical course in our patient in the 2nd decade is of particular interest. In addition, Fabry disease occurring with gluten-sensitive enteropathy, a very rare co-existence, is emphasized.     

阴囊Paget''s病的诊断与治疗(附6例报告)

目的:提高阴囊Paget's病的认识,探讨该病的诊断与治疗方法.方法:回顾性分析6例阴囊Paget's病的诊治资料,4例病灶仅累及单侧阴囊者行包括病灶的扩大切除术;1例复发者行双侧病灶扩大切除加左侧睾丸、附睾及精索切除,然后行腹部带蒂皮瓣及大腿游离皮瓣修补术;1例切除病灶后行阴囊底部旋转横行皮瓣阴茎成形术.结果:6例手术效果满意,术后病理检查均报告为阴囊Paget's病.结论:本病临床较为罕见,极易与阴囊湿疹混淆,需要经病理检查确诊.治疗上以外科手术治疗为主.     

脊柱包虫病的诊断与治疗

目的:探讨脊柱包虫病的诊断及治疗方法。方法:1990年10月～2010年10月手术治疗脊柱包虫病患者9例,病程为3个月～12年。临床表现为午后低热、腰背疼痛和腰背部包块,术前脊髓神经功能Frankel分级A级1例,B级2例,C级2例,D级4例。包虫病8项免疫试验均为阳性。术前均行影像学检查,病变单纯累及胸椎2例(T8、T9 1例,T11、T12 1例),腰椎2例(L3 1例,L1、L2、L3 1例),胸腰段4例(T12、L1 2例,T12、L1、L2 2例),骶椎1例。X线片、CT检查误诊为椎体结核5例、转移瘤2例、脊索瘤1例、腰大肌脓肿1例。MRI检查7例诊断为脊柱包虫病;2例病变单纯累及腰椎者缺乏囊中囊典型信号改变,误诊为脊柱转移瘤。均行椎管减压病灶清除植骨内固定术,其中病变单纯累及胸椎的2例与骶骨1例行后路全椎板切除减压,2例腰椎病变者行椎板间开窗椎管减压,4例胸腰段病变者行脊柱侧前方入路经横突椎管减压。7例术前诊断脊柱包虫病者术前口服抗包虫药阿苯达唑2～3周,所有患者术后继续服用阿苯达唑3个月。结果:手术时间210～330min,平均260min;术中失血量170～470ml,平均300ml。7例术前MRI检查诊断为脊柱包虫病者术后病理诊断为脊柱细粒棘球蚴病,2例术前MRI误诊为脊柱转移瘤者术后病理诊断为腰椎泡状棘球蚴病。随访1～10年,平均4.6年。1例腰椎泡状棘球蚴病患者术后8个月植骨未愈合,经理疗、促骨生长药物治疗,1年后复查X线片显示骨性愈合;其余8例患者植骨区骨性融合,愈合时间为5～8.5个月,平均7个月。末次随访患者脊髓神经功能Frankel分级改善6例,无变化3例。随访期间无复发。结论:MRI检查对脊柱细粒棘球蚴病有诊断价值,对腰椎泡状棘球蚴病易误诊,后者的确诊有赖于病理检查;脊柱包虫病在药物治疗的基础上采用手术治疗可取得较好疗效。