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.     

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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。     

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.     

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。     

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.     

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.     

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。     

炎性肠病45例手术治疗及疗效评价

目的 探讨炎性肠病的手术治疗方法 及疗效评估.方法 回顾性分析近6年经外科手术治疗的45例炎性肠病患者的临床资料.结果 急诊手术16例,Crohn病9例,溃疡性结肠炎(UC)7例.择期手术29例,Crohn病4例,UC 25例.其中13例Crohn病患者行小肠部分切除6例,内瘘者行小肠及结肠部分切除、吻合术1例,回肠穿...     

A case of unilateral renal cystic disease

Unilateral renal cystic disease (URCD) is a distinct entity that is one of the renal cystic diseases. The clinical importance of URCD is to make a differential diagnosis from autosomal dominant polycystic kidney disease (ADPKD), multicystic dysplastic kidney, multilocular cystic renal neoplasm, and simple cysts. To confirm the diagnosis and to rule out asynchronous ADPKD requires long-term follow up, especially in younger patients.     

克罗恩病外科治疗85例分析

目的总结分析肠道克罗恩病（CD）的外科治疗策略。方法对1980—2005年收治的85例CD病人资料进行回顾性分析。结果近年来CD病人呈增多趋势，术前确诊率为23．53％。肠梗阻（25．88％）、右下腹包块（回盲部肿物，10．59％）、盲肠癌（12．94％）、急性阑尾炎（4．71％）是术前误诊的几大主要原因。手术方式以右半结肠切除术（37．65％），小肠部分切除（21．18％）为主，内、外瘘及肛周CD行外科处理预后良好。结论手术仍是目前肠道CD的重要治疗手段；手术方式依病变部位和并发症类型不同而有差异，术式选择和规范化手术操作是亟待解决的问题。     

小剂量罗哌卡因鞍麻在肛肠病合并心血管病手术中的应用

为观察小剂量罗哌卡因鞍麻在肛肠病合并心血管病手术中的应用，将300例肛肠病合并心血管病患者随机分为观察组和对照组各150例，即小剂量罗哌卡因鞍麻组和局麻组。结果显示，小剂量罗哌卡因鞍麻镇痛完全，明显降低了麻醉及手术的并发症，提高了手术的安全性。结果表明，小剂量罗哌卡因鞍麻是适用于肛肠病合并心血管病患者手术的麻醉方法。     

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.     

住院的慢性肾脏病患者营养状态及其相关因素分析

目的了解住院的慢性肾脏病（chronic kidney disease,CKD）患者营养状态,并分析其相关因素。方法统计分析402例住院的CKD患者的体质量指数（body mass index,BMI）与血红蛋白（Hb）、血清白蛋白（Alb）、血脂各组份之间的关系。结果402例慢性肾脏病患者中营养不良发生率7．46％,超重和肥胖发生率41．3％。超重组的Hb、Alb、TP、TG、ApoB高于BMI正常组,肥胖组的Hb、Alb、TP、TG、TC、ApoB、LDL-C高于BMI正常组,差异有统计学意义（P〈0．05）。营养不良组血红蛋白、总蛋白低于非营养不良组。结论住院的CKD患者有较高的超重和肥胖发生率,营养不良患者常合并贫血。     

A 15-year review of children with Kawasaki's Syndrome having general anesthesia or deep sedation

BACKGROUND: Children with Kawasaki's syndrome (KS), also known as Kawasaki's disease or 'mucocutaneous lymph node syndrome', have approximately 20-25% incidence of developing coronary artery aneurysms (CAA), stenosis or obliteration if not appropriately diagnosed and treated. In addition some children have myocarditis, pericardial effusions and/or cardiac arrhythmias during the acute phase of KS. Even with current treatment protocols, 2-4% will still be at risk of coronary artery pathology and the long-term implications regarding future coronary artery disease are unknown. Many of these children present for surgical or diagnostic procedures requiring general anesthesia or deep sedation. Only sporadic case reports have been published on the anesthetic experiences of such patients. METHODS: With Institutional Review approval, we reviewed the medical records of all children with discharge diagnosis of KS from 1985 to 2000 for those receiving general anesthesia or deep sedation. Data abstracted from the medical records included information on any surgical procedures performed any time after onset of KS symptoms, type of anesthetic, perioperative monitoring and presence or absence of operative or perioperative complications. RESULTS: A total of 178 children with KS were identified of whom 47 (26.4%) received either general anesthesia (34) or deep sedation (13). There were no deaths; one child developed congestive heart failure in the immediate postoperative period associated with KS myocarditis. Five (15%) of those having general anesthesia initially were either not diagnosed as having KS or had no preoperative cardiac evaluations. None of the children having general anesthesia had ST segment analysis, invasive monitoring or troponin measurements perioperatively. CONCLUSIONS: The high incidence of serious myocardial complications attributable to KS reported in the pediatric literature is rarely noted in the anesthesia literature. We feel there is a potential for more serious perioperative complications among KS children, although we can only speculate why complications are not more frequently encountered. Anesthetists involved in pediatric services are encouraged to consider KS in their diagnosis of children presenting with febrile illnesses with rashes and to consider the possibility of KS myocardial compromise if they encounter unexpected deterioration perioperatively. Preoperative ultrasound examination and perioperative monitoring (e.g. ST segment analysis and troponin measurements) for myocardial compromise are encouraged if KS is suspected.     

Potential Advantages and Limitations of Applying the Chronic Kidney Disease Classification to Kidney Transplant Recipients

The National Kidney Foundation (NKF) Kidney Disease Outcomes Quality Initiative (K/DOQI) classification of Chronic Kidney Disease (CKD) characterizes patients by their level of kidney function and includes kidney transplant recipients (KTRs). Most KTRs have stage > or = 3 CKD (estimated glomerular filtration rate < 60 mL/min/1.73 m2) and may benefit from aggressive CKD care. Recent modifications to the K/DOQI CKD classification reflect the recognition of KTRs as a unique subset of CKD patients in whom the presentation, progression and implications of CKD may vary from those in nontransplant CKD populations. Currently, there is limited information about how adopting the CKD classification in KTRs will influence clinical management and outcomes. Appropriately designed studies are needed to develop transplant-specific CKD treatment recommendations, and to ensure patient, health provider and payer acceptance of the continued need for aggressive CKD care after transplantation. Education and implementation strategies will be required to ensure appropriate integration of the CKD classification and treatment guidelines into existing posttransplant care programs. The CKD classification thus represents an exciting potential strategy to improve clinical outcomes that should be adopted, further studied and modified to incorporate considerations unique to KTRs.     

Review of cinacalcet hydrochloride in the management of secondary hyperparathyroidism

Cinacalcet is the first Food and Drug Administration-approved calcimimetic for the treatment of secondary hyperparathyroidism in dialysis patients. It is effective in improving control of parathyroid hormone, serum calcium, phosphorus, and calcium-phosphorus product. The calcium-lowering effect of cinacalcet overcomes the limitations of standard therapy associated hypercalcemia. There is evidence to suggest that cinacalcet has important clinical implications, which extend beyond its relevance in the treatment of secondary hyperparathyroidism. This review summarizes the evidence regarding the role of cinacalcet in the treatment of secondary hyperparathyroidism, disrupted bone mineral metabolism, cardiovascular disease, and mortality. In addition, the cost implications of cinacalcet are briefly explored.