Hypertrophic cardiomyopathy masquerading as infiltrative restrictive cardiomyopathy and refractory congestive failure-resolution with catheter ablation of atrial flutter – A case report

We report a case of a young male presenting as Restrictive cardiomyopathy, refractory heart failure and syncope due to typical right atrial flutter complicating hypertrophic cardiomyopathy.Successful catheter ablation of the flutter promptly ameliorated the congestive failure with resolution of restrictive physiology.     

中西医一体化防治增生性瘢痕的研究现状

皮肤瘢痕是一个巨大的医学问题,每年约有1亿患者产生瘢痕。瘢痕的预防/治疗在很大程度上还没有得到满足的临床需求。瘢痕的形成通常是皮肤创伤的病理结果。而增生性瘢痕(hypertrophic scar,HTS)常发生在人深部损伤后。HTS通常被认为是组织收缩和过度的细胞外基质沉积的结果。肌成纤维细胞作为主要由成纤维细胞分化而来的效应细胞,在HTS的病理生理中起着至关重要的作用。多种生长因子、炎症因子参与了HTS发生的过程。目前,随着对HTS的深入探索和临床研究,各种创造性和有效的治疗方法正在萌芽。本文就HTS中西医形成机制研究进展进行综述,并针对其病理生理特点,对现有的中西医综合治疗方法进行综述。此外,我们进一步展望更适合的HTS中西医综合治疗的特色与方法。     

Distinct osteogenic mechanisms of bones of distinct origins

Mammalian bones have three distinct origins (paraxial mesoderm, lateral plate mesoderm, and neural crest) and undergo two different modes of formation (intra-membranous and endochondral). Bones derived from the paraxial mesoderm and lateral plate mesoderm mainly form through the endochondral process. During this process, hypertrophic chondrocytes play a vital role in inducing both osteogenesis and angiogenesis. One of the essential osteogenic factors secreted from hypertrophic chondrocytes is Indian hedgehog (Ihh). In contrast, bones derived from the neural crest mainly form through the intramembranous pro-cess and do not require Ihh. Thus, depending on their origin, bones have distinct signaling properties, which need to be considered in the research and application of bone biology.Presented at the 18th Annual Research Meeting of the Japanese Orthopaedic Association, Kitakyushu, Japan, October 17, 2003     

Characterization of T-cell subsets infiltrating post-burn hypertrophic scar tissues

In this study, skin-infiltrating cells were characterized in both the active and remission phases of post-burn hypertrophic scar biopstes. Immunohistochemistry examination of active phase samples showed an abundant presence of Langerhans cells, T cells, macrophages, a low presence of natural killer cells and the lack of B lymphocytes. In active hypertrophic scars T lymphocytes infiltrate deep into the superficial dermis and are also observed in the epidermis: CD3⁺ cells were present at about 222±107 per 0.25 mm². In particular the analysis of lymphocyte subpopulations showed that CD4⁺ T cells predominate in the dermis as well as in the epidermis of active hypertrophic scars whereas CD8⁺ cells were less well represented (CD4/CD8 ratio is 2.06). This distribution was also shown in remission phase samples and in normotrophic scar specimens, although the lymphocyte number was significantly lower. Approximately 70 per cent of T lymphocytes present in the tissue involved in active phase hypertrophic scar samples were activated (positive with anti-HLA-DR and IL-2 receptor antibodies) which is significantly higher than remission phase hypertrophic and normotrophic scars, in which positivity was 40 and 38 per cent, respectively. Upon activation, the lesional lymphocytes release several cytokines, locally and transiently, that interact with specific receptors in response to different stimulation. Central to the immune hypothesis of hypertrophic scars is that some of the T-cell lymphokines act on keratinocytes, fibroblasts and other cell types to induce changes characteristic of these scars. The presence and close proximity of activated T lymphocytes and antigen-presenting cells of various phenotypes in both the epidermis and dermis of hypertrophic tissues provides strong circumstantial evidence of a local immune response. However, the manner in which T cells achieve and maintain their activated state in hypertrophic tissues in not yet known, and both antigen-dependent and independent mechanisms may contribute.     

The Domain of Hypertrophic Chondrocytes in Growth Plates Growing at Different Rates

In this study, we tested the hypotheses that (a) both the domain volume (volume of the cell and the matrix it has formed) and matrix volume of juxtametaphyseal hypertrophic chondrocytes in the growth plate is tightly controlled, and that (b) the domain volume of juxtametaphyseal hypertrophic chondrocytes is a strong determinant of the rate of bone length growth. We analyzed the rate of bone length growth (oxytetracycline labeling techniques) and nine stereologic and kinetic parameters related to the juxtametaphyseal chondrocytic domain in the proximal and distal radial and tibial growth plates of 21- and 35-day-old rats. The domain volume increased with increasing growth rates, independent of the location of the growth plate and the age of the animal. Within age groups, the matrix volume per cell increased with increasing growth rates, but an identical growth plate had the same matrix volume per cell in 21- and 35-day-old rats. The most suitable regression model (R ²= 0.992) to describe the rate of bone length growth included the mean volume of juxtametaphyseal hypertrophic chondrocytes and the mean rate of cell loss/cell proliferation. This relationship was independent of the location of the growth plate and the age of the animal. The data suggest that the domain volume of juxtametaphyseal hypertrophic chondrocytes, as well as the matrix volume produced per cell, may be tightly regulated. In addition, the volume of juxtametaphyseal hypertrophic chondrocytes and the rate of cell loss/rate of cell proliferation may play the most important role in the determination of the rate of bone length growth. Received: 2 December 1996 / Accepted: 24 March 1997     

巢蛋白(nestin)在瘢痕疙瘩和增生性瘢痕中的表达

目的探讨巢蛋白（nestin）在瘢痕疙瘩和增生性瘢痕中的表达。方法采用免疫组织化学技术方法检测8例正常皮肤、7例扁平瘢痕、8例瘢痕疙瘩、8例增生性瘢痕中nestin^＋细胞，计数分析nestin^＋细胞在瘢痕疙瘩和增生性瘢痕中的表达。结果①nestin在8例正常皮肤组织中表皮基底细胞和棘细胞、汗腺、毛囊、皮脂腺、血管内皮细胞和7例成纤维细胞的胞浆中表达。nestin在扁平瘢痕、瘢痕疙瘩、增生性瘢痕组织中表皮基底细胞和棘细胞、血管内皮细胞和成纤维细胞的胞浆及残留的汗腺、毛囊、皮脂腺中表达。②瘢痕疙瘩和增生性瘢痕组织中nestin^＋表皮细胞、nestin^＋成纤维细胞和nestin^＋血管内皮细胞的数量明显高于正常皮肤和扁平瘢痕（P〈0．05），而正常皮肤与扁平瘢痕组织中nestin^＋细胞的表达差异无显著性（P〉0．05）。结论巢蛋白在瘢痕疙瘩和增生性瘢痕表皮、成纤维细胞和血管内皮细胞中表达增高提示瘢痕疙瘩和增生性瘢痕的过度增生可能与巢蛋白相关。     

腹腔镜下幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄50例报告

目的总结腹腔镜下幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄的成功经验.方法2001年4月～2004年4月,应用腹腔镜下幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄50例,年龄12～90 d,平均35 d.分别在左、右上腹各置入3 mm trocar,左侧trocar置入无损伤抓钳夹近幽门处胃壁,右侧trocar先后置入伸缩式幽门肌切开刀、剥离器和幽门分离钳,完成幽门环肌切开术.结果腹腔镜下完成手术48例,中转开腹2例,其中1例为术中发现幽门前瓣膜症,1例为幽门黏膜损伤,经开腹修补痊愈.手术时间15～45 min,平均25 min.术后6 h拔胃管,开始喂奶.3～5 d出院.42例术后随访3～6个月,平均4.5月,生长发育均恢复正常.结论丰富的开腹手术经验、熟练的腹腔镜操作技术、术中良好的麻醉和合适的手术器械是完成腹腔镜下幽门环肌切开术的保障.     

Idiopathic hypertrophic cranial pachymeningitis: a case report

Hypertrophic pachymeningitis is a rare fibrosing inflamatory process involving dura mater and tentorium. In this report we are presenting contrast enhanced MRI findings of an unusual case of pachymeningitis which presented with a periorbital mass due to dural sinuses occlusion and retrograde filling of periorbital veins through superior sagittal sinus.     

肿泡眼切开法重睑成形术式探讨

目的　探讨肿泡眼单睑患者的理想重睑成形术式。方法　针对肿泡眼单睑的解剖特点 ,对常规切开法重睑成形术加以改进。通过较窄重睑线设计 ,去除适量松弛皮肤 ,去除臃肿眶隔脂肪及肥厚眼轮匝肌、睑板前结缔组织 ,改善重睑形态。结果　本组患者共 30 8例 ,随访 15 0例 ,随访时间 3～ 12个月。其中 12 3例效果满意 ,19例效果一般 ,8例不甚满意。结论　对于肿泡眼患者可在切开法重睑成形术的基础上 ,将松弛皮肤、眶隔脂肪、眼轮匝肌及睑板前结缔组织适量去除 ,术后重睑形态自然流畅     

Genomic structure and eight novel exonic polymorphisms of the human N-cadherin gene

 Analysis of the detailed genomic structure of human N-cadherin revealed that the 16-exon gene is more than 72 kb in length and that it consists of a mosaic of exons. Five repeated cadherin domains, a transmembrane domain, and a cytoplasmic domain are encoded by exons 4 to 13, 13 and 14, and 14 to 16, respectively. A search for molecular variants in the entire coding region in 96 Japanese individuals resulted in the identification of eight sequence polymorphisms including three CCT- or GCC-type trinucleotide repeat polymorphisms adjacent to the initiation codon and five other novel single-nucleoticle polymorphisms (SNPs) in the coding region. Three of the five SNPs accompanied an amino acid substitution: Ala118Thr, Ala826Thr, and Asn845Ser. Knowlege of the fine gene structure and eight novel polymorphisms will be useful for the genetic study of the role of N-cadherin in diseases involving cell adhesion in the brain and in cardiomyocytes. Received: January 23, 2002 / Accepted: March 12, 2002