畸形性骨炎一例报道

通过1例Paget's骨病的治疗分析,探讨其病因、诊断及治疗。Paget's骨病临床表现为慢性骨痛、骨畸形,血碱性磷酸酶持续增高,X线片及骨扫描有特异性改变。Paget's骨病在我国罕见,排除相关疾病时应想到该病的可能,治疗以双膦酸盐为主,首选唑来膦酸。     

畸形性骨炎伴低血钙、低血磷一例

报道1例畸形性骨炎,又称Paget骨病(Paget's disease,PD)伴低血钙、低血磷病例。患者是围绝经期女性,以骨痛,血碱性磷酸酶增高以及骨骼影像学特征性病变明确诊断为Paget骨病,用唑来膦酸治疗后骨痛减轻,X线片提示多骨损害有好转。但是,不同于典型Paget骨病,该患者血钙、血磷及维生素D水平降低,甲状旁腺素增高。本文结合文献复习,总结分析伴钙、磷代谢异常Paget骨病的临床表现、实验室检查和治疗情况,以期提高临床医师对本病的认识、为临床诊治积累更多经验。     

多种代谢性骨病对患者生活质量的影响

代谢性骨病是由多种原因引起骨骼矿盐代谢紊乱、骨转换异常或骨骼矿化障碍的全身性骨骼疾病,临床可以有进行性骨痛、多发骨折、骨骼畸形等多种表现。这类疾病严重影响患者机体健康状况,也会对患者生活质量带来不利影响。本文就近期关于多种代谢性骨病对患者生活质量影响的研究进展进行综述。     

贵州省贵定县乐邦乡严重骨畸形病病例调查研究

对贵州省贵定县乐邦乡村民罹患以骨骼严重畸形为特征的疾病病例研究结果显示，病家生活燃煤，敞灶烘烤之玉米，辣椒及室内贮存之谷子铝和氟含量显著高于对照。患者尿中铝和氟含量亦较对照高。２８例骨畸形病人中仅有２１例患轻或中度氟斑牙。病人骨Ｘ光片呈骨软化症，无氟骨症骨周组织异常骨化。三例严重骨畸形病人髂骨活检，现人骨铝含量显著增高，但骨氟含量无明显增加，骨形态计量学测量显示，病人骨损特征为骨发育障碍，成骨过程     

二膦酸盐在成骨不全疾病中的应用

成骨不全是一种以脆骨症为典型特征的遗传性结缔组织疾病,该病最严重的后果为骨脆弱所导致的频繁骨折、继发性骨骼畸形,90%的成骨不伞是I型胶原基因突变所致.临床研究证实,二膦酸盐可抑制破骨细胞活性,增加骨密度、降低骨折发生率、改善患者生活质量,是目前内科对成骨不全治疗的有效手段.本文就二膦酸盐的作用机制、治疗方法、疗效、不良反应及存在的问题等予以综述.     

糖尿病与骨质疏松症

骨质疏松症是指骨组织中单位体积内骨量明显减少,导致骨强度降低和微结构损毁,因造成骨骼不同程度的病理改变而出现的一个症候群。常有腰背部、髋部的疼痛和身长的缩短以及驼背等骨骼畸形,严重者在稍遇外力时极易发生骨折。骨     

罕见黏多糖贮积症ⅣA型致病基因GALNS突变谱和骨骼表型

目的 分析罕见黏多糖贮积症ⅣA型(mucopolysaccharidosis typeⅣA,MPSⅣA)家系患者的临床特点,行致病基因突变研究,探讨MPSⅣA患者骨骼表型与基因突变谱的特点.方法 纳入以步态异常、骨骼畸形为主要表现的3个家系4例患者,评估患者临床表现、骨转换生化指标、骨密度、骨影像学及MPS相关酶学指标...     

强直性脊柱炎胸腰段后凸畸形外科治疗的研究进展

强直性脊柱炎(AS)是一种主要累及中轴骨骼的慢性炎症性风湿性疾病,可引起脊柱韧带进行性成骨性改变,最终导致僵硬后凸,常导致胸腰段、颈胸段后凸畸形,以胸腰段后凸畸形多见.对于AS导致的脊柱胸腰段后凸畸形以手术截骨矫形为主要治疗手段.该文就胸腰段后凸畸形外科治疗的研究进展作一综述.     

脱氧雪腐镰刀菌烯醇致小鼠骨骼畸形模型的建立

目的制作脱氧雪腐镰刀菌烯醇(DON)胎鼠模型,观察DON对小鼠的致骨骼畸形作用和胚胎毒性,为进一步研究DON致畸的机理提供试验平台。方法将妊娠小鼠分为溶剂对照组和DON实验组,每组6只孕鼠。实验组小鼠于孕期第7~10 d连续腹腔注射DON,每天2.5 mg/kg体重,总剂量为10 mg/kg体重。溶剂对照组注射30%丙二醇。妊娠第18 d解剖孕鼠,实验组随机取31只胎鼠,溶剂对照组取63只胎鼠,去除软组织,进行骨骼、软骨双重染色,镜下观察骨骼发育情况。结果实验组31只胎鼠均出现多发性骨骼畸形,其中胸骨畸形率为90.32%,胸椎畸形率90.32%,四肢畸形率为54.84%。溶剂对照组胎鼠胸骨畸形率为9.52%,未见其他骨畸形。结论DON可致胎鼠多发性骨骼畸形;DON小鼠模型可用于骨骼先天性畸形研究。     

影像学与骨质疏松症关系的研究进展

骨质疏松症是一种以骨强度降低致使机体罹患骨折危险I生增加为特征的骨骼疾病,以骨量减少、骨组织微结构退化为特征,致使骨的脆性增加以及易于发生骨折的一种全身性骨骼疾病。     

State of knowledge of helminth fauna of freshwater fishes of Poland

A total 89 fish and lamprey species has been recorded from Polish freshwater habitats. Twenty-seven of them (30.3%) have not been surveyed for parasitic helminthes. Some of the latter fishes are either rare or not easily accessible. Other live only in specific habitats in scattered localities. An important obstacle for studying parasite faunas of some fishes may be their status on an endangered species. Among the non-surveyed fishes, are those which have been relatively recently introduced to Poland or migrated there on their own. The present paper attempts to review all hitherto not studied helminthologically fish species, their habitats, localities and current protection status.     

高血压降压治疗目标的再认识

根据传统的高血压水平的定义,1993年WHO高血压治疗指南提出血压控制目标为<140/90mm Hg(1mm Hg=0.133kPa),但是并非所有患者都必须将血压降至同一水平,而应根据患者情况进行个体化治疗。Framingham进行的一项长达10～12年的心血管事件研究发现,第5年后,正常上限血压[收缩压(SBP     

Lack of correlation of degree of villous atrophy with severity of clinical presentation of coeliac disease

BACKGROUND AND AIM: Both the clinical presentation and the degree of mucosal damage in coeliac disease vary greatly. In view of conflicting information as to whether the mode of presentation correlates with the degree of villous atrophy, we reviewed a large cohort of patients with coeliac disease. PATIENTS AND METHODS: We correlated mode of presentation (classical, diarrhoea predominant or atypical/silent) with histology of duodenal biopsies and examined their trends over time. RESULTS: The cohort consisted of 499 adults, mean age 44.1 years, 68% females. The majority had silent coeliac disease (56%) and total villous atrophy (65%). There was no correlation of mode of presentation with the degree of villous atrophy (p=0.25). Sixty-eight percent of females and 58% of males had a severe villous atrophy (p=0.052). There was a significant trend over time for a greater proportion of patients presenting as atypical/silent coeliac disease and having partial villous atrophy, though the majority still had total villous atrophy. CONCLUSIONS: Among our patients the degree of villous atrophy in duodenal biopsies did not correlate with the mode of presentation, indicating that factors other than the degree of villous atrophy must account for diarrhoea in coeliac disease.