结节性黄瘤病并发主动脉瓣上狭窄和主动脉瓣关闭不全

报告1例结节性黄瘤病并发主动脉瓣上狭窄、主动脉瓣关闭不全.患儿男,13岁.全身多发性黄色结节13年,2年前因出现心悸、胸闷就诊.皮损组织病理检查符合黄瘤病,结合临床表现和心脏彩色多普勒超声检查,诊断为结节性黄瘤病并发主动脉瓣七狭窄和主动脉瓣关闭不全.     

内分泌、代谢、营养障碍性皮肤病

20061368多功能电离子手术治疗机治疗睑黄瘤40例/曾奇生(福建建瓯市立医院皮肤科)∥皮肤病与性病.-2005,27(4).-35选治疗仪短火档,电压6V,炭化病变组织。结果术后7~10天脱痂,1月后恢复正常皮肤;痊愈35例,显效5例,有效率100%,均未出现明显疤痕;随访半年至1年,2例复发。(张江安)20061369结节性黄瘤病并发主动脉瓣狭窄1例/赵娟(中南大学湘雅医院皮肤科),谭琦,颜兰香∥临床皮肤科杂志.-2006,35(3).-140~141男,12岁。全身多发黄色结节12年,皮肤组织病理示真皮浅层大量泡沫细胞。心脏彩色多普勒超声诊断为主动脉瓣狭窄。治疗以降脂为主,饮食要求…     

儿童中心脏手术后的脱发

已有报道术后脱发是麻醉的一种少见并发症,主要发生于长时间手术后的成年人。报告3例婴儿发生于心脏手术后的脱发,其中2例术后2年尚无头发再生。因此认为其脱发是永久性的。 例1。男,因主动脉瓣闭锁不全,二尖瓣狭窄以及肺动脉高压,16个月时行二尖瓣置换术,2岁时又进行了主动脉瓣及二尖瓣置换。第2次手术后7天,发现头顶上方有一结     

论包皮粘连及包皮口狭窄在现代男科学中的地位

胚胎9周时阴茎头已发育并完全裸露,而包皮迟至12周时才形成并逐渐向阴茎头远端方向生长,至14周时才覆盖整个阴茎头并与阴茎头粘连。这种生理性粘连一直持续至出生时,伴或不伴有包皮口狭窄,随着阴茎发育多在17岁前自然消失,如不消失,就变为病理性;因炎症等因素成人原本已翻动自如的包皮可以再次发生包皮口狭窄及包皮粘连,此属于病理性。作为一门正在蓬勃发展中的学科,现代男科学应在前所未有的高度上重视包皮粘连及包皮口狭窄的诊断及处理,以期阴茎的结构及功能得到良好的保障。本文对包皮粘连及包皮口狭窄加以分析,对有关研究进展加以综述,认为这两者在现代男科学中具有重要的地位。     

自制“灯笼式”后尿道吻合器在后尿道狭窄术中的应用

目的探索提高后尿道狭窄治疗效果的方法。方法选取2016年10月至2018年12月中国人民解放军第九二四医院泌尿男科诊治的35例后尿道狭窄患者作为研究对象。年龄范围12岁～72岁,平均45岁。全部病例均经膀胱尿道造影确诊,后尿道狭窄长度1 cm～4.5cm,平均2.5cm。3例合并前尿道狭窄。经会阴径路行倒"Y"切口,分离球部及阴囊部尿道,在狭窄处切断尿道,充分切除后尿道瘢痕组织,采用自制"灯笼式"后尿道吻合器在显示器直视下用3-0可吸收线间断行后尿道吻合6针～8针。27例行狭窄后尿道切除后端端吻合,8例采用阴囊纵隔带蒂皮瓣卷管成尿道后与后尿道行端端吻合。结果手术时间50 min～120min,平均75min,术后2周～3周拔出导尿管,随访2个月～28个月,平均14个月。35例患者术后排尿通畅,不需尿道扩张,最大尿流率15ml/S。一次性手术治愈率94%。2例术后再狭窄,1例行尿道扩张治愈,1例再次行狭窄部切除吻合治愈。结论采用自制"灯笼式"后尿道狭窄吻合器行后尿道吻合术治疗瘢痕性后尿道狭窄,吻合方便,手术时间短,成功率高,是一种较好的手术方式。     

TPPA阳性“冠心病”患者梅毒血清试验与超声心动图研究

目的分析近2年来TPPA阳性的“冠心病”患者的梅毒血清试验、心脏彩超和胸片资料,为这类患者在诊断心血管梅毒上提供思路和方法。方法对以“冠心病”相关诊断入院的心内、心外科及急诊抢救中心的TPPA阳性患者进行梅毒血清复检,并对患者进行了心脏彩超检查。结果132例TPPA阳性患者,RPR、TPHA阳性者分别47(35.61%)例,130(98.49%)例。心脏彩超检查,132例主动脉(AA)根径(32.2±4.2)mm,65例升主动脉(Aao)内径(37.9±5.1)mm,均大于正常值,并且AA根径平均值与TPPA阴性冠心病患者相比有统计学差异;主动脉瓣钙化49(37.13%)例,主动脉瓣返流52(39.40%)例,较老年性AV退行性变差异有显著性。胸片示:主动脉增宽86.61%(97/112),主动脉钙化41.08%(46/112)。RPR阳性/阴性患者的AA根径、Aao内径差异无统计学意义;但主动脉瓣钙化及返流有统计学差异。结论对不能提供相关病史的TPPA、TPHA同时阳性患者,应考虑患者既往感染过梅毒螺旋体。即使RPR阴性,且未见其他阳性体征,仅AA及Aao稍大及主动脉瓣钙化、主动脉瓣返流的冠心病患者,也应对可能潜在的梅毒性损害给予重视,并在合适时机实施规范化治疗及复查。     

“屏障”及“加固”技术在复杂尿道阴道瘘修复中的应用（附7例报告）

目的：总结大阴唇球海绵体脂肪垫（Martius瓣）“屏障”及阴道壁“加固”技术在复杂尿道阴道瘘修复中的作用。方法：回顾2009年1月-2012年3月采用Martius瓣“屏障”技术及阴道壁“加固”术修复复杂尿道阴道瘘7例。年龄12—35岁,平均22岁;产伤1例,车祸致下尿路生殖道损伤6例,瘘孔大小2．0～3．Ocm;合并尿道狭窄2例、后尿道闭锁2例、阴道狭窄3例。探讨了补瘘与处理合并症时机,介绍了手术方法及术后处理。结果：7例全部1次修复成功,随访6～24个月。5例排尿通畅,最大尿流率12～20ml／s,2例后尿道闭锁者术后出现尿线变细,扩张一次后治愈。1例膀胱颈闭锁者术后有轻度压力性尿失禁。阴道狭窄者均满意康复。结论：Martius瓣“屏障”及阴道壁“加固”术可明显提高复杂尿道阴道瘘修复成功率。     

皮肤病综合征

20 0 12 664　白塞氏病 35例临床分析 /李楠 (广东省医院皮肤科 )∥实用医学杂志 .- 2 0 0 1,17( 1) .- 70～ 71男 2 1例、女 14例 ,年龄 4～ 67岁。 68.60 %以口腔溃疡为首发症状 ;74 .30 %以外阴溃疡为首发症状 ;有眼部损害者占 31.4 0 % ;皮肤损害为首发症状 8例 ,为多形性 ;57.14 %针刺反应阳性 ;关节损害为 4 2 .85% ;8例出现心脏损害 ,主要表现为主动脉瓣关闭不全 ,4例出现血管炎损害。作者强调 ,白塞病可能发生心脏损害 ,且可以是本病的主要死因。参 6　 (马勇 )2 0 0 12 665　白塞病与 EB病毒感染及干扰素治疗观察/马百芳 (山东德…     

儿童白色萎缩1例

报告1例儿童白色萎缩。患者,女,12岁。因双侧踝内侧反复出现红斑、瘀斑、溃疡、疼痛5个月就诊。组织病理检查:真皮浅层小血管增生,管腔狭窄,管腔内有透明血栓形成,少量红细胞外溢,血管壁有纤维蛋白样物质沉积。诊断:白色萎缩。     

梅毒血清阳性冠心病患者发生特异性心血管损害的危险性

为探讨TPPA阳性患者潜在的心血管损害,对TPPA阳性患者进行梅毒血清复检,以TPPA、TPHA均阳性者为TPPA阳性组.同时收集同期住院的TPPA阴性的无其它血管、瓣膜合并症的冠心病患者为对照组,两组均行心脏彩超检查.结果:TPPA阳性组146例,其中RPR阳性58例(39.7%).心脏彩超检查,主动脉根径(AA-d)(33.3±3.9)mm,升主动脉内径(Aao-d)(35.2±5.2)mm;主动脉瓣钙化62例(42.4%),主动脉瓣返流63例(43.2%),较对照组有显著性差异;且发生主动脉瓣钙化的危险是TPPA阴性患者的2.0倍;Aao-d值升高和性别也是重要的发病危险因素.TPPA组中RPR阳性/阴性患者的上述资料差异无统计学意义.TPPA阳性患者尽管无病史支持,不能诊断为梅毒或梅毒性心脏病,但也不能忽视可能潜在的梅毒损害.     

Familial hypercholesterolemia (Type IIb) in a child: a case report with interesting features

Familial hypercholesterolemia (FH) is the most commonly recognized disorder of lipoprotein metabolism in childhood. We report a case of FH in a 5-year-old boy with onset of jaundice since birth, and multiple planar, tuberous, palmar and intertrigenous xanthomas covering the trunk and limbs. His total cholesterol was 590 mg/dl and triglycerides were 171 mg/dl. Echocardiography revealed mild aortic stenosis as a result of premature atherosclerosis. He was diagnosed with homozygous FH, and is reported here because of the interesting clinical features.     

Autosomal recessive form of congenital cutis laxa: more than the clinical appearance

Congenital cutis laxa is an uncommon disorder of generalized elastolysis. The clinical picture is characterized by inelastic, loose, hanging skin that gives the appearance of premature aging. The disease is inherited most commonly in a severe autosomal recessive form, or as a relatively benign, autosomal dominant form. There is often systemic organ involvement in patients with the autosomal recessive form. Cardiopulmonary abnormalities are common and mainly determine the prognosis and life expectancy. Pulmonary emphysema, cor pulmonale, and right-sided heart failure generally caused by pulmonary disease are often seen in infancy. Various cardiovascular abnormalities including aortic aneurysm, pulmonary artery multiple branch stenosis have been reported in patients with this form of congenital cutis laxa. We report a 10-month-old boy with the autosomal recessive form of congenital cutis laxa who had pulmonary valve stenosis. To the best of our knowledge, this is the first case of this association to be reported in the English language literature. We also emphasize the systemic complications that may be associated with congenital cutis laxa.     

Alkaptonuria

A 69-year-old woman presented with a 30-year history of lower back and large joint pain of the hips and shoulders. On examination blue-grey, pigmented macules were present over the cartilaginous portions of the ears and on the sclera. Past medical history included aortic stenosis. Urine homogentisic acid level was elevated, which is diagnostic for alkaptonuria. Alkaptonuria is an autosomal recessive disorder that results in deficiency of homogentisic acid oxidase and in the accumulation of homogentisic acid in connective tissue. Disease can result in blue-grey pigmentation of the cartilage, sclerae, face, and hands as well as severe arthropathy and cardiac valve disease. Treatment is limited at this time. Promising early reports of the use of nitisinone have prompted ongoing trials of this therapeutic agent.     

Phacomatosis pigmentokeratotica: a follow-up report documenting additional cutaneous and extracutaneous anomalies

Abstract:  This is a follow-up report on a boy with phacomatosis pigmentokeratotica. At the age of 10 years he had, in addition to a sebaceous nevus and a speckled lentiginous nevus, multiple lesions of a collagen nevus localized on the chin and in the lumbar area. On the left shoulder, a small telangiectatic spot was present within the area of the speckled lentiginous nevus. Moreover, hemiatrophy of the left-hand side of the body and hyperhidrosis of the left lumbar area were noted. At the age of 16, the lesions of his collagen nevus had considerably enlarged and showed an arrangement along Blaschko lines. Additional pinhead-sized vascular lesions were noted, with preponderance within the area of the speckled lentiginous nevus in the left scapular region and on his left leg. Moreover, the boy had developed severe arterial hypertension since the age of 13. Angiographic examination showed an aortic stenosis that reached from the aortic arch down to the origin of the renal arteries, necessitating a surgical intervention. From this follow-up report we conclude that phacomatosis pigmentokeratotica may be associated with other cutaneous abnormalities such as linear connective tissue nevus of the collagen type and multiple pinhead-sized angioma-like lesions superimposed on the speckled lentiginous nevus. The associated defects of the large vessels may belong to the component of Schimmelpenning syndrome representing one "half" of phacomatosis pigmentokeratotica, rather than being part of the speckled lentiginous syndrome that forms the other "half" of this twin-spot phenotype.     

Poikiloderma,tendon contracture and pulmonary fibrosis: a new autosomal dominant syndrome?

Members of two generations of a South African family have a unique syndrome comprising poikiloderma, tendon contractures and progressive pulmonary fibrosis. The condition is clinically important as the skin changes, which involve the face, have considerable cosmetic impact, while lung involvement is potentially lethal in adulthood. Skin manifestations which facilitate diagnosis include facial telangiectasia, mottled hypo- and hyperpigmentation, papules and epidermal atrophy. The scalp, facial and body hair are fine and scanty. The tendon contractures lead to progressive digital flexion deformities and abnormalities of the ankles and feet, with disturbance of gait. Pulmonary involvement manifests as progressive dyspnoea. Pedigree data are compatible with an autosomal dominant mode of transmission. Poikiloderma of Weary is characterized by linear sclerotic and fibrous bands and not tendon contractures and is not associated with potentially lethal pulmonary fibrosis. Rather than name this disorder a variant of Weary syndrome, it might be prudent to use as an umbrella title one composed by Weary himself: 'hereditary sclerosing poikiloderma' (HSP), under which variants such as HSP Weary type, HSP with cardiac involvement (aortic stenosis described as inconsistently associated with Weary syndrome) and HSP with tendon/pulmonary involvement (current family) may be classified. The manifestations in this family differ from other poikilodermata and, to the best of our knowledge, have not been previously documented.     

冠心病合并梅毒男性患者超声心动图的特征表现

目的探讨冠心病合并梅毒男性患者超声心动图的特征表现。方法选取2017年3月至2019年7月汉川市人民医院收治的32例有梅毒感染史或确诊梅毒且合并冠心病男性患者作为研究对象。将这32例患者纳入冠心病合并梅毒组。选取同期汉川市人民医院住院的64例单纯冠心病男性患者纳入单纯冠心病组。两组均接受超声心动图的检查。比较两组超声心动图的心脏主动脉根部直径、升主动脉直径和主动脉瓣反流、主动脉钙化的差异。结果冠心病合并梅毒组心脏主动脉钙化、心脏主动脉瓣反流的几率明显比单纯冠心病组高,升主动脉直径明显比单纯冠心病组大,差异具有统计学意义(P<0.05);两组心脏主动脉根部直径比较,差异无统计学意义(P>0.05);冠心病合并梅毒组两亚组的心脏超声相关指标比较,差异无统计学意义(P>0.05)。结论冠心病合并梅毒男性患者会增加心脏主动脉钙化、心脏主动脉瓣反流、升主动脉直径变大的几率,应给予密切监测。     

主动脉夹层32例临床分析

目的初步探讨主动脉夹层的急诊诊治思路，提高急诊医师对主动脉夹层的认识，提高早期诊断率。方法分析32例主动脉夹层患者的临床表现及相关辅助检查，总结其临床特点。结果主动脉夹层多以剧烈疼痛为主要表现，胸背腰疼痛为主，大部分有高血压病史；CT、MRI是确诊本病的重要手段。结论急诊工作中对有剧烈胸背腰疼痛，伴血压升高者，应进行CTA检查，以明确有无主动脉夹层。     

Klippel–Trenaunay and Sturge–Weber syndrome with extensive Mongolian spots, hypoplastic larynx and subglottic stenosis

An East Indian infant with Klippel–Trenaunay syndrome and Sturge–Weber syndrome is described. This infant also had giant Mongolian spots, hypoplastic larynx and subglottic stenosis. The association of hypoplastic larynx and subglottic stenosis with Klippel–Trenaunay syndrome and Sturge–Weber syndrome has not been reported previously.     

比较肋软骨和摇门式肌皮瓣在喉气管狭窄重建中的效果

目的回顾性分析第四军医大学唐都医院l983年～2001年收治的喉气管狭窄114例,比较肋软骨和摇门式肌皮瓣2种移植物在喉气管狭窄重建中的效果。方法随机采用肋软骨移植喉气管重建术53例,摇门式胸舌骨肌皮瓣移植喉气管重建术55例,复合肋软骨肌皮瓣移植喉气管重建术6例。2种方法都先行喉气管裂开,黏膜下切除瘢痕。肋软骨组:取一侧带软骨膜肋软骨1块,移植于喉气管狭窄区前壁。T形管支撑。肌皮瓣组:在喉气管狭窄区喉气管旁作一双蒂胸舌骨肌皮瓣,翻转180°,肌皮瓣移植于喉气管狭窄前壁,T形管支撑。6例合并颈前壁大瘘,采用复合肋软骨和肌皮瓣移植。结果肋软骨移植组46例(占86.8%)一次手术治愈。7例失败,其中3例伤口感染软骨坏死,2例T形管顶端损伤黏膜形成新狭窄,2例瘢痕体质,再狭窄。肌皮瓣移植组50例(占90.9%)一次手术治愈。5例失败中3例皮瓣下陷入气管腔,2例喉气管内又长瘢痕狭窄。复合肋软骨肌皮瓣移植组6例,4例治愈,2例软骨坏死失败。随访1～10年,肌皮瓣组l例术前病因不清者,术后3年再狭窄,其余疗效巩固。结论2种移植物对喉气管狭窄重建术都有较高治愈率。颈部皮肤血供差者慎用肋软骨移植,颈部皮下脂肪厚者不宜用摇门式肌皮瓣移植,颈前壁有大瘘口者宜采用复合肋软骨肌皮瓣移植。     

Combined functional and anatomical diagnostic endpoints for assessing arteriovenous fistula dysfunction

Failure of arteriovenous fistulas(AVF) to mature and thrombosis in matured fistulas have been the major causes of morbidity and mortality in hemodialysis patients. Stenosis, which occurs due to adverse remodeling in AVFs, is one of the major underlying factors under both scenarios. Early diagnosis of a stenosis in an AVF can provide an opportunity to intervene in a timelymanner for either assisting the maturation process or avoiding the thrombosis. The goal of surveillance strategies was to supplement the clinical evaluation(i.e., physical examination) of the AVF for better and earlier diagnosis of a developing stenosis. Surveillance strategies were mainly based on measurement of functional hemodynamic endpoints, including blood flow(Q a) to the vascular access and venous access pressure(VAP). As the changes in arterial pressure(MAP) affects the level of VAP, the ratio of VAP to MAP(VAPR = VAP/MAP) was used for diagnosis. A Q a 400-500 m L/min or a VAPR 0.55 is considered sign of significant stenosis, which requires immediate intervention. However, due to the complex nature of AVFs, the surveillance strategies have failed to consistently detect stenosis under different scenarios. VAPR has been primarily developed to detect outflow stenosis in arteriovenous grafts, and it hasn't been successful in accurate diagnosis of outflow lesions in AVFs. Similarly, AVFs can maintain relatively high blood flow despite the presence of a significant outflow stenosis and thus, Q a has been found to be a better predictor of only inflow lesions. Similar shortcomings have been reported in the detection of functional severity of coronary stenosis using diagnostic endpoints that were based on either flow or pressure. This limitation has been associated with the fact that both pressure and flow change in the presence of a stenosis and thus, hemodynamic diagnostic endpoints that employ only one of these parameters are inherently prone to inaccuracies. Recent attempts have resulted in development of new diagnostic endpoints that can combine the effects of pressure and flow. These new hemodynamic diagnostic endpoints have shown to be better predictors of functional severity of lesions as compared to either flow or pressure based counterparts. In this review article, we discussed the advantages and limitations of current functional and anatomical diagnostic endpoints in AVFs.